CCZ1

Chr 7

CCZ1 homolog, vacuolar protein trafficking and biogenesis associated

The protein acts as a guanine nucleotide exchange factor for RAB7 in concert with MON1A, promoting vesicle-mediated transport within cells. Mutations cause autosomal recessive spastic paraplegia with intellectual disability and seizures, typically presenting in early childhood. The gene shows minimal constraint against loss-of-function variants.

OMIMResearchSummary from RefSeq, UniProt
LOEUF 0.92
Clinical SummaryCCZ1
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
Some data sources returned errors (1)

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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.92LOEUF
pLI 0.000
Z-score 1.83
OE 0.57 (0.360.92)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
-0.19Z-score
OE missense 1.04 (0.931.17)
199 obs / 191.5 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.57 (0.360.92)
00.351.4
Missense OE1.04 (0.931.17)
00.61.4
Synonymous OE1.13
01.21.6
LoF obs/exp: 12 / 21.1Missense obs/exp: 199 / 191.5Syn Z: -0.87

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

CCZ1 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

3D Protein StructureAlphaFold

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

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Clinical Literature
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