{
  "vercy": "1.0-draft",
  "publication": {
    "status": "research-draft",
    "adjudicationStatus": "unreviewed",
    "publishableCanonical": false,
    "generatedAt": "2026-09-12T10:01:19Z",
    "providers": [
      "Codex"
    ],
    "breadth": "recalled by Codex without web access - no source was read",
    "missingProviders": [],
    "pass": 2,
    "cost": {
      "grok": {
        "seconds": 19.3,
        "error": "Reading additional input from stdin...\nOpenAI Codex v0.153.4\n--------\nworkdir: R:\\02_PROJECTS\\02_Meta_Models_Platforms\\Ver.cy\\current\\thing-registry-backlog\nmodel: gpt-6-astra\nprovider: openai\napproval: never\nsandbox: read-only\nreasoning effort: none\nreasoning summaries: none\nsession id: 01a0950f-41d6-7ed2-9c37-be00ba0b7211\n--------\nuser\nDescribe what is already known about one registered thing. Answer as JSON only, no prose around it.\n\nThing: Glucosylceramidase beta\nSense to describe: (none recorded)\nDomain code: PHY.MAT\nAlso known as: (none)\n\n\nContext for this batch of 1001 things:\n# Batch 0",
        "usd": 0.0,
        "recall": true
      },
      "codex": {
        "seconds": 79.8,
        "error": "Reading additional input from stdin...\nOpenAI Codex v0.153.4\n--------\nworkdir: R:\\02_PROJECTS\\02_Meta_Models_Platforms\\Ver.cy\\current\\thing-registry-backlog\nmodel: gpt-6-astra\nprovider: openai\napproval: never\nsandbox: read-only\nreasoning effort: none\nreasoning summaries: none\nsession id: 01a0950f-41e0-73a3-9bd7-b237869eac95\n--------\nuser\nYou are drafting a Vercy meta-model for one registered thing. Answer as JSON only, no prose around it.\n\nThing: Glucosylceramidase beta\nRegistry id: vr.tr.glucosylceramidase-beta\nPlane / domain: PHY / PHY.MAT\nRegistry definition: (none recorded)\nNames folded in"
      }
    },
    "enrichment": {
      "pass": 3,
      "engine": "claude",
      "producedAt": "2026-10-06T12:00:00Z",
      "ingestedAt": "2026-10-06T08:56:03+00:00",
      "fields": [
        "agent_conduct",
        "ethics",
        "failure_modes_and_hazards",
        "owners",
        "visual_identification"
      ]
    },
    "gate": {
      "passed": true,
      "version": "1.0",
      "at": "2026-10-07T10:10:30+00:00",
      "evidence": [
        "completeness 1.00",
        "sense: Wikidata description",
        "2 live sources",
        "review by codex: pass"
      ]
    }
  },
  "metaModel": {
    "id": "THING-Q420038",
    "registryId": "vr.tr.glucosylceramidase-beta",
    "name": "Glucosylceramidase beta",
    "version": "0.1.0-research.1",
    "entryKind": "thing",
    "family": "Thing Registry",
    "domain": [
      "PHY.MAT"
    ],
    "status": "research-draft"
  },
  "canonicalUrl": "https://ver.cy/models/thing/q420038/",
  "model": {
    "registry_id": "vr.tr.glucosylceramidase-beta",
    "name": "Glucosylceramidase beta",
    "purpose": "Enable an agent to identify glucosylceramidase beta, assess its molecular integrity and catalytic competence, and select justified characterization or handling actions.",
    "definition": "Glucosylceramidase beta is an enzyme that hydrolyses glucosylceramide into glucose and ceramide, usually referring to the lysosomal enzyme encoded by human GBA1.",
    "scope_statement": "This model provisionally treats vr.tr.glucosylceramidase-beta as the GBA1-associated lysosomal enzyme, owning its protein identity, molecular forms, catalytic behavior, cellular context and preparation quality; the registry's intended species and gene-versus-protein boundary remain unresolved.",
    "in_scope": [
      "Species, protein accession, sequence version and relationship to the encoding gene",
      "Precursor processing, mature protein, sequence variants and glycosylation",
      "Glucosylceramide hydrolysis and experimentally supported substrate specificity",
      "Lysosomal localization, trafficking and activity-supporting interactions",
      "Assay-specific activity, molecular integrity and preparation suitability"
    ],
    "out_of_scope": [
      "The GBA1 genomic locus, inheritance and transcriptional regulation as independently modeled entities",
      "GBA2 and other glucosidases as separate enzyme identities",
      "Glucosylceramide, ceramide and glucose as independently modeled substances",
      "Gaucher disease, Parkinson's disease and patient diagnosis or prognosis",
      "Therapeutic formulations, prescribing, dosing and manufacturing authorization"
    ],
    "distinguishing_features": [
      "Require a species-qualified protein sequence or accession: human UniProt P04062 identifies lysosomal acid glucosylceramidase associated with GBA1. [UniProt GBA1](https://www.uniprot.org/uniprotkb/P04062/entry)",
      "Test hydrolysis of glucosylceramide to glucose and ceramide; activity on a generic artificial beta-glucoside alone does not establish the intended enzyme identity. [UniProt GBA1](https://www.uniprot.org/uniprotkb/P04062/entry)",
      "Distinguish GBA1 from GBA2 using molecular identity and cellular localization because both can hydrolyze glucosylceramide. [UniProt GBA2](https://www.uniprot.org/uniprotkb/Q9HCG7/entry)",
      "Distinguish the protein from its encoding DNA by the evidence object: protein sequence, peptides or intact protein versus a genomic or transcript accession. [NCBI GBA1](https://www.ncbi.nlm.nih.gov/gene/2629)",
      "For recombinant or therapeutic material, compare construct sequence, processing and preparation identifiers before treating it as equivalent to the endogenous enzyme."
    ],
    "characteristics": [
      {
        "name": "Species-qualified protein identity",
        "kind": "relation",
        "unit_or_values": "Organism taxonomy identifier, protein accession and sequence version",
        "why_it_matters": "Prevents an unqualified enzyme name from silently selecting human protein or merging distinct orthologs."
      },
      {
        "name": "Molecular form",
        "kind": "category",
        "unit_or_values": "Precursor, mature endogenous protein, recombinant construct or characterized variant",
        "why_it_matters": "Sequence processing and construct differences affect comparisons of mass, residues and function."
      },
      {
        "name": "Sequence variation",
        "kind": "relation",
        "unit_or_values": "Reference sequence, residue-numbering convention and explicit substitutions or deletions",
        "why_it_matters": "Makes variant claims interpretable across precursor and mature-protein numbering."
      },
      {
        "name": "Glycosylation profile",
        "kind": "measurement",
        "unit_or_values": "Site occupancy percentage and glycoform relative abundance under a named method",
        "why_it_matters": "Characterizes preparation differences that sequence identity alone cannot resolve."
      },
      {
        "name": "Catalytic specific activity",
        "kind": "measurement",
        "unit_or_values": "µmol product/min/mg enzyme, with substrate, pH, temperature and additives specified",
        "why_it_matters": "Supports functional comparison only when assay conditions and normalization are compatible."
      },
      {
        "name": "Assay environment",
        "kind": "measurement",
        "unit_or_values": "pH, temperature in °C, substrate concentration in µM and lipid or detergent composition",
        "why_it_matters": "Separates changes in enzyme competence from changes in substrate presentation or reaction conditions."
      },
      {
        "name": "Cellular localization",
        "kind": "state",
        "unit_or_values": "Lysosomal localization demonstrated, mislocalized, mixed localization or unmeasured",
        "why_it_matters": "Connects protein abundance with access to the cellular compartment where function is being evaluated."
      },
      {
        "name": "Interaction context",
        "kind": "relation",
        "unit_or_values": "Measured association or experimental supplementation with LIMP-2, saposin C and specified membranes",
        "why_it_matters": "Records trafficking and activation context needed to interpret cellular or reconstituted measurements."
      },
      {
        "name": "Preparation integrity",
        "kind": "state",
        "unit_or_values": "Intact, fragmented, aggregated, mixed or uncharacterized, with method and date",
        "why_it_matters": "Prevents nominal protein concentration from being mistaken for usable enzyme concentration."
      },
      {
        "name": "Retained activity",
        "kind": "measurement",
        "unit_or_values": "Percentage of initial activity after specified storage or handling conditions",
        "why_it_matters": "Supports evidence-based decisions about reuse and comparison of stored aliquots."
      }
    ],
    "affordances": [
      "Resolve an enzyme label against species-qualified protein records and flag ambiguous mappings.",
      "Compare endogenous and recombinant forms using sequence, processing and glycosylation evidence.",
      "Select and interpret glucosylceramide hydrolysis assays with appropriate specificity controls.",
      "Investigate reduced activity through separate measurements of abundance, localization and catalytic competence.",
      "Accept, recharacterize or exclude an enzyme preparation from a stated experiment using documented quality criteria."
    ],
    "agent_conduct": {
      "may": [
        "Describe the enzyme, its reaction and its gene, citing biochemical sources.",
        "Report its link to Gaucher disease and approved enzyme therapies from official sources.",
        "Record the identity, activity and storage of a research enzyme preparation."
      ],
      "must_not": [
        "Infer or disclose a person's genetic variants or disease risk from data without consent.",
        "Advise a particular patient on enzyme replacement or other treatment.",
        "Overstate research links to other diseases as established fact.",
        "Present a research-grade preparation as fit for human use."
      ],
      "requires_human": [
        "Any use of genetic test results about a person.",
        "Releasing an enzyme preparation for clinical use."
      ]
    },
    "ethics": {
      "considerations": [
        "Variants in its gene affect disease risk, making genetic data about it sensitive.",
        "Treatments for rare diseases are costly, raising questions of fair access."
      ],
      "affected_parties": [
        "People with Gaucher disease and families",
        "Carriers of gene variants",
        "Researchers"
      ]
    },
    "owners": {
      "steward": "Nobody owns the enzyme; holders of preparations and marketing authorization holders of therapies answer for them.",
      "master_systems": [
        "Protein and gene databases",
        "National medicines registers for therapeutic forms"
      ]
    },
    "visual_identification": [
      "Not visible; identified by sequence, activity assays and labels.",
      "Research and therapeutic preparations carry different labelling."
    ],
    "failure_modes_and_hazards": [
      "Loss of activity from poor storage gives false results.",
      "Misinterpreted genetic findings cause needless anxiety or false reassurance."
    ],
    "what_it_is_for": [
      "Glucosylceramidase beta is encountered in studies of lysosomal lipid metabolism and the breakdown of glucosylceramide.",
      "The human enzyme is investigated in research on Gaucher disease and biological pathways associated with Parkinson disease.",
      "Purified preparations and cellular samples are used to study enzyme identity, folding, localization and catalytic activity."
    ],
    "standards_and_regulation": [
      "The Enzyme Commission classification maintained by the International Union of Biochemistry and Molecular Biology assigns glucosylceramidase to EC 3.2.1.45."
    ]
  },
  "sources": [
    {
      "id": "wikidata",
      "title": "Wikidata item Q420038: Glucosylceramidase beta",
      "url": "https://www.wikidata.org/wiki/Q420038",
      "url_status": "live",
      "what_it_supports": "identity and sense of the item",
      "checked_by": "wikidata-api"
    },
    {
      "id": "wikipedia-en",
      "title": "Wikipedia: Glucocerebrosidase",
      "url": "https://en.wikipedia.org/wiki/Glucocerebrosidase",
      "url_status": "live",
      "what_it_supports": "general description of the item",
      "checked_by": "wikidata-api sitelink"
    }
  ],
  "structure": {
    "bundles": [
      {
        "id": "enzyme-identity",
        "name": "Enzyme identity",
        "description": "Resolve which biological protein the registry name denotes.",
        "rationale": "The name lacks a species and can be confused with a gene label or a shared catalytic activity.",
        "layers": [
          {
            "id": "registry-referent",
            "name": "Registry referent",
            "description": "Establish the intended entity and its authoritative identifiers.",
            "findings": [
              {
                "id": "protein-gene-boundary",
                "name": "Protein versus encoding gene",
                "description": "Human GBA1 is a gene record linked to lysosomal acid glucosylceramidase protein records; the registry must identify which entity it owns. [NCBI GBA1](https://www.ncbi.nlm.nih.gov/gene/2629)",
                "questions": [
                  {
                    "text": "Does the originating registry record denote the enzyme protein, its encoding gene or an enzyme activity class?",
                    "kind": "boundary",
                    "id": "protein-gene-boundary-q01"
                  },
                  {
                    "text": "Which original identifier and source establish that interpretation?",
                    "kind": "provenance",
                    "id": "protein-gene-boundary-q02"
                  }
                ]
              }
            ]
          },
          {
            "id": "homolog-discrimination",
            "name": "Homolog discrimination",
            "description": "Separate the intended enzyme from proteins sharing its reaction.",
            "findings": [
              {
                "id": "gba1-gba2-discrimination",
                "name": "GBA1 and GBA2 discrimination",
                "description": "GBA2 is a non-lysosomal glucosylceramidase with overlapping hydrolytic activity, making reaction identity insufficient for assigning GBA1. [UniProt GBA2](https://www.uniprot.org/uniprotkb/Q9HCG7/entry)",
                "questions": [
                  {
                    "text": "Which species-qualified accession or sequence identifies the protein under examination?",
                    "kind": "definition",
                    "id": "gba1-gba2-discrimination-q01"
                  },
                  {
                    "text": "What molecular or experimental evidence excludes GBA2 and other contributing glucosidases?",
                    "kind": "boundary",
                    "id": "gba1-gba2-discrimination-q02"
                  }
                ]
              }
            ]
          }
        ]
      },
      {
        "id": "molecular-form",
        "name": "Molecular form",
        "description": "Describe the particular processed, modified or engineered enzyme being assessed.",
        "rationale": "An enzyme name alone does not determine the sequence, glycoform or physical integrity of a preparation.",
        "layers": [
          {
            "id": "sequence-processing",
            "name": "Sequence and processing",
            "description": "Anchor residue-level interpretation to a defined protein form.",
            "findings": [
              {
                "id": "reference-and-maturation",
                "name": "Reference and maturation",
                "description": "The human reference entry includes isoforms and processing into a mature form; sequence and numbering conventions must accompany comparisons. [UniProt GBA1](https://www.uniprot.org/uniprotkb/P04062/entry)",
                "questions": [
                  {
                    "text": "Which accession version and isoform define the reference sequence?",
                    "kind": "provenance",
                    "id": "reference-and-maturation-q01"
                  },
                  {
                    "text": "Which residues are present in the measured mature protein or recombinant construct?",
                    "kind": "measurement",
                    "id": "reference-and-maturation-q02"
                  },
                  {
                    "text": "Are reported substitutions numbered against the precursor, mature chain or another documented reference?",
                    "kind": "definition",
                    "id": "reference-and-maturation-q03"
                  }
                ]
              }
            ]
          },
          {
            "id": "glycoform-and-integrity",
            "name": "Glycoform and integrity",
            "description": "Characterize differences beyond the encoded amino acid sequence.",
            "findings": [
              {
                "id": "measured-protein-form",
                "name": "Measured protein form",
                "description": "Record glycosylation, intact mass, fragmentation and aggregation as preparation-specific observations rather than universal constants.",
                "questions": [
                  {
                    "text": "What glycosylation profile and intact mass were measured for this preparation?",
                    "kind": "measurement",
                    "id": "measured-protein-form-q01"
                  },
                  {
                    "text": "What fraction is intact protein, and what evidence detects fragments or aggregates?",
                    "kind": "measurement",
                    "id": "measured-protein-form-q02"
                  }
                ]
              }
            ]
          }
        ]
      },
      {
        "id": "catalysis-and-assay",
        "name": "Catalysis and assay",
        "description": "Connect reported activity to the substrate, reaction and measurement conditions.",
        "rationale": "A positive glucosidase signal must be attributable to the intended enzyme and relevant substrate.",
        "layers": [
          {
            "id": "substrate-and-products",
            "name": "Substrate and products",
            "description": "Define the chemical transformation being evaluated.",
            "findings": [
              {
                "id": "glucosylceramide-hydrolysis",
                "name": "Glucosylceramide hydrolysis",
                "description": "The reference enzyme hydrolyzes glucosylceramides to glucose and ceramides; assays should identify the substrate species and measured product. [UniProt GBA1](https://www.uniprot.org/uniprotkb/P04062/entry)",
                "questions": [
                  {
                    "text": "Which glucosylceramide molecular species or artificial substrate is supplied?",
                    "kind": "definition",
                    "id": "glucosylceramide-hydrolysis-q01"
                  },
                  {
                    "text": "Which product or substrate-depletion measurement demonstrates turnover?",
                    "kind": "measurement",
                    "id": "glucosylceramide-hydrolysis-q02"
                  },
                  {
                    "text": "What evidence supports extending an artificial-substrate result to glucosylceramide hydrolysis?",
                    "kind": "boundary",
                    "id": "glucosylceramide-hydrolysis-q03"
                  }
                ]
              }
            ]
          },
          {
            "id": "activity-attribution",
            "name": "Activity attribution",
            "description": "Make activity values comparable and distinguish enzyme signal from interference.",
            "findings": [
              {
                "id": "conditioned-specific-activity",
                "name": "Conditioned specific activity",
                "description": "Treat activity as a conditional measurement requiring defined substrate presentation, normalization and controls.",
                "questions": [
                  {
                    "text": "What pH, temperature, substrate concentration, lipid composition and detergent conditions produced the activity value?",
                    "kind": "measurement",
                    "id": "conditioned-specific-activity-q01"
                  },
                  {
                    "text": "How were background signal, other glucosidases and nonlinearity with time or enzyme amount excluded?",
                    "kind": "measurement",
                    "id": "conditioned-specific-activity-q02"
                  },
                  {
                    "text": "Should an incompatible assay be repeated before comparing this enzyme with another preparation?",
                    "kind": "action",
                    "id": "conditioned-specific-activity-q03"
                  }
                ]
              }
            ]
          }
        ]
      },
      {
        "id": "lysosomal-function",
        "name": "Lysosomal function",
        "description": "Connect enzyme competence with delivery and substrate access in cells.",
        "rationale": "Reduced cellular turnover may reflect trafficking or activation context as well as the enzyme molecule itself.",
        "layers": [
          {
            "id": "delivery-and-localization",
            "name": "Delivery and localization",
            "description": "Assess whether the enzyme reaches the relevant compartment.",
            "findings": [
              {
                "id": "limp2-trafficking-context",
                "name": "LIMP-2 trafficking context",
                "description": "LIMP-2 participates in GCase trafficking to lysosomes; localization evidence should accompany claims about cellular enzyme function. [GCase trafficking study](https://pubmed.ncbi.nlm.nih.gov/26094596/)",
                "questions": [
                  {
                    "text": "What imaging or fractionation evidence demonstrates lysosomal localization in the studied cells?",
                    "kind": "measurement",
                    "id": "limp2-trafficking-context-q01"
                  },
                  {
                    "text": "Was LIMP-2 abundance or association measured when investigating altered localization?",
                    "kind": "measurement",
                    "id": "limp2-trafficking-context-q02"
                  }
                ]
              }
            ]
          },
          {
            "id": "activation-and-substrate-access",
            "name": "Activation and substrate access",
            "description": "Represent the environment supporting lipid-substrate turnover.",
            "findings": [
              {
                "id": "saposin-c-context",
                "name": "Saposin C context",
                "description": "Saposin C supports glucocerebrosidase function, and defects in this context can accompany impaired lipid degradation. [Saposin C and LIMP-2 deficiency study](https://pmc.ncbi.nlm.nih.gov/articles/PMC11204053/)",
                "questions": [
                  {
                    "text": "Is saposin C endogenous, supplemented, deficient or unmeasured in this system?",
                    "kind": "measurement",
                    "id": "saposin-c-context-q01"
                  },
                  {
                    "text": "How is glucosylceramide presented to the enzyme in the cellular or reconstituted membrane system?",
                    "kind": "definition",
                    "id": "saposin-c-context-q02"
                  },
                  {
                    "text": "Which experiment would distinguish deficient enzyme catalysis from inadequate activation or substrate access?",
                    "kind": "action",
                    "id": "saposin-c-context-q03"
                  }
                ]
              }
            ]
          }
        ]
      },
      {
        "id": "preparation-and-use",
        "name": "Preparation and use",
        "description": "Determine whether a particular enzyme preparation supports its intended experimental use.",
        "rationale": "Expression history, contaminants and loss of activity can invalidate otherwise well-designed enzyme experiments.",
        "layers": [
          {
            "id": "preparation-provenance",
            "name": "Preparation provenance",
            "description": "Trace the enzyme material and its suitability for a stated experiment.",
            "findings": [
              {
                "id": "lot-and-construct-evidence",
                "name": "Lot and construct evidence",
                "description": "Link each preparation to its source, expression system, construct, purification record and measured contaminants.",
                "questions": [
                  {
                    "text": "Which lot, expression host, construct and purification record identify this material?",
                    "kind": "provenance",
                    "id": "lot-and-construct-evidence-q01"
                  },
                  {
                    "text": "Could co-purifying glucosidases, residual inhibitors or formulation components alter the intended readout?",
                    "kind": "boundary",
                    "id": "lot-and-construct-evidence-q02"
                  },
                  {
                    "text": "Which acceptance criteria must this preparation meet for the proposed biochemical or cellular experiment?",
                    "kind": "action",
                    "id": "lot-and-construct-evidence-q03"
                  }
                ]
              }
            ]
          },
          {
            "id": "handling-and-retention",
            "name": "Handling and retention",
            "description": "Track whether handling preserves measurable enzyme performance.",
            "findings": [
              {
                "id": "activity-after-storage",
                "name": "Activity after storage",
                "description": "Base storage and reuse decisions on preparation-specific instructions and measured retained activity.",
                "questions": [
                  {
                    "text": "What buffer, temperature, storage duration and freeze-thaw history apply to this aliquot?",
                    "kind": "provenance",
                    "id": "activity-after-storage-q01"
                  },
                  {
                    "text": "How much activity remains relative to a baseline measured with the same assay?",
                    "kind": "measurement",
                    "id": "activity-after-storage-q02"
                  },
                  {
                    "text": "Does the aliquot meet the stated reuse criteria, or does it require recharacterization or replacement?",
                    "kind": "action",
                    "id": "activity-after-storage-q03"
                  }
                ]
              }
            ]
          }
        ]
      },
      {
        "id": "evidence-and-external-alignment",
        "name": "Evidence and external alignment",
        "description": "What the world already says about this thing, gathered so the model can be checked against it.",
        "rationale": "A model that cannot be lined up against existing standards, identifiers and practice cannot be adopted by anyone who already uses them.",
        "layers": [
          {
            "id": "reported-evidence",
            "name": "Reported evidence",
            "description": "Findings from the breadth pass, kept separate from the structural claims.",
            "findings": [
              {
                "id": "evidence-confidence-notes",
                "name": "Check these first",
                "description": "Recalled without web access and unsourced; every item is a lead to verify.",
                "evidence": [
                  "The registry supplies neither organism nor sense; confirm whether it means human GBA1 protein, an ortholog or the broader catalytic activity.",
                  "GBA1 and GBA2 encode distinct enzymes, not interchangeable isoforms of one protein.",
                  "Activity reference intervals depend on specimen, substrate and assay; no universal numerical range is supplied."
                ],
                "questions": [
                  {
                    "id": "evidence-confidence-notes-q01",
                    "text": "Which of these check these first hold for the sense of Glucosylceramidase beta this model covers, and on what evidence?",
                    "kind": "provenance"
                  }
                ]
              },
              {
                "id": "evidence-kinds",
                "name": "Kinds and varieties",
                "description": "Recalled without web access and unsourced; every item is a lead to verify.",
                "evidence": [
                  "Lysosomal glucosylceramidase beta, encoded by GBA1",
                  "Non-lysosomal glucosylceramidase beta, encoded by GBA2"
                ],
                "questions": [
                  {
                    "id": "evidence-kinds-q01",
                    "text": "Which of these kinds and varieties hold for the sense of Glucosylceramidase beta this model covers, and on what evidence?",
                    "kind": "provenance"
                  }
                ]
              },
              {
                "id": "evidence-identifiers",
                "name": "Identifiers and schemes",
                "description": "Recalled without web access and unsourced; every item is a lead to verify.",
                "evidence": [
                  {
                    "scheme": "IUBMB Enzyme Commission number",
                    "value_or_pattern": "EC 3.2.1.45",
                    "note": "Classification of glucosylceramidase catalytic activity; it does not uniquely identify a species or protein sequence."
                  },
                  {
                    "scheme": "HGNC gene symbol",
                    "value_or_pattern": "GBA1",
                    "note": "Human gene encoding lysosomal glucosylceramidase beta; the gene and its protein product are distinct entities."
                  },
                  {
                    "scheme": "UniProtKB accession",
                    "value_or_pattern": "P04062",
                    "note": "Human lysosomal glucosylceramidase beta."
                  }
                ],
                "questions": [
                  {
                    "id": "evidence-identifiers-q01",
                    "text": "Which of these identifiers and schemes hold for the sense of Glucosylceramidase beta this model covers, and on what evidence?",
                    "kind": "provenance"
                  }
                ]
              },
              {
                "id": "evidence-standards-and-regulation",
                "name": "Standards and regulation",
                "description": "Recalled without web access and unsourced; every item is a lead to verify.",
                "evidence": [
                  "International Union of Biochemistry and Molecular Biology enzyme nomenclature provides the catalytic classification."
                ],
                "questions": [
                  {
                    "id": "evidence-standards-and-regulation-q01",
                    "text": "Which of these standards and regulation hold for the sense of Glucosylceramidase beta this model covers, and on what evidence?",
                    "kind": "provenance"
                  }
                ]
              },
              {
                "id": "evidence-real-world-use",
                "name": "Real-world use",
                "description": "Recalled without web access and unsourced; every item is a lead to verify.",
                "evidence": [
                  "Measurement of enzyme activity contributes to laboratory diagnosis of Gaucher disease.",
                  "Recombinant forms of the lysosomal enzyme are used in enzyme replacement therapy for Gaucher disease.",
                  "Research on sphingolipid metabolism, lysosomal function and mechanisms associated with Parkinson disease."
                ],
                "questions": [
                  {
                    "id": "evidence-real-world-use-q01",
                    "text": "Which of these real-world use hold for the sense of Glucosylceramidase beta this model covers, and on what evidence?",
                    "kind": "provenance"
                  }
                ]
              },
              {
                "id": "evidence-failure-modes-and-hazards",
                "name": "Failure modes and hazards",
                "description": "Recalled without web access and unsourced; every item is a lead to verify.",
                "evidence": [
                  "Deficient lysosomal enzyme activity can cause Gaucher disease, with accumulation of glucosylceramide and related metabolites.",
                  "Some pathogenic variants impair protein folding, stability or delivery to lysosomes.",
                  "GBA1 variants are associated with increased Parkinson disease risk, but do not determine that an individual will develop it.",
                  "Activity measurements can be misleading if sample integrity, substrate specificity and assay conditions are not controlled."
                ],
                "questions": [
                  {
                    "id": "evidence-failure-modes-and-hazards-q01",
                    "text": "Which of these failure modes and hazards hold for the sense of Glucosylceramidase beta this model covers, and on what evidence?",
                    "kind": "provenance"
                  }
                ]
              },
              {
                "id": "evidence-neighbours",
                "name": "Neighbouring kinds and how to tell them apart",
                "description": "Recalled without web access and unsourced; every item is a lead to verify.",
                "evidence": [
                  {
                    "name": "Glucosylceramide",
                    "difference": "A lipid substrate of the enzyme, rather than the enzyme itself."
                  },
                  {
                    "name": "GBA1",
                    "difference": "The gene encoding the human lysosomal enzyme, rather than its protein product."
                  },
                  {
                    "name": "Galactosylceramidase",
                    "difference": "Hydrolyses galactosylceramide, whose sugar is galactose rather than glucose."
                  },
                  {
                    "name": "Glucosylceramide synthase",
                    "difference": "Produces glucosylceramide by transferring glucose to ceramide, rather than hydrolysing it."
                  }
                ],
                "questions": [
                  {
                    "id": "evidence-neighbours-q01",
                    "text": "Which of these neighbouring kinds and how to tell them apart hold for the sense of Glucosylceramidase beta this model covers, and on what evidence?",
                    "kind": "provenance"
                  }
                ]
              }
            ]
          }
        ]
      }
    ]
  },
  "openQuestions": [
    "Does the registry's originating identifier denote human GBA1 protein, its gene, a cross-species enzyme concept or the broader glucosylceramidase activity class?",
    "Does an existing Vercy world model already own this concept, requiring a registry link instead of a second model?",
    "Which orthologs, isoforms, engineered variants and therapeutic enzyme forms belong within this entry's authoritative granularity?",
    "Which assay conditions and specificity controls should establish comparable activity measurements for the intended uses?",
    "Which preparation-specific evidence establishes acceptable glycosylation, purity, storage conditions and retained activity?"
  ],
  "statistics": {
    "bundles": 6,
    "layers": 11,
    "findings": 17,
    "questions": 33
  }
}
