TBC1D32

Chr 6AR

TBC1 domain family member 32

Also known as: ALHSA, BROMI, C6orf170, C6orf171, OFD9, RP100

This protein is required for high-level sonic hedgehog responses in the developing neural tube and controls primary cilium structure by coordinating assembly of the ciliary membrane and axoneme. Mutations cause autosomal recessive ciliopathies including Alsahan-Harris syndrome, orofaciodigital syndrome IX, and retinitis pigmentosa 100. These conditions primarily affect craniofacial development, limb formation, and retinal function due to disrupted ciliary signaling pathways.

GeneReviewsOMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismARLOEUF 0.903 OMIM phenotypes
Clinical SummaryTBC1D32
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
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ClinVar Variants
48 unique Pathogenic / Likely Pathogenic· 224 VUS of 403 total submissions
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GeneReview available — TBC1D32
Authoritative clinical overview · Recommended first read
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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.90LOEUF
pLI 0.000
Z-score 2.23
OE 0.72 (0.570.90)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
0.04Z-score
OE missense 1.00 (0.931.06)
622 obs / 624.6 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.72 (0.570.90)
00.351.4
Missense OE1.00 (0.931.06)
00.61.4
Synonymous OE1.11
01.21.6
LoF obs/exp: 52 / 72.5Missense obs/exp: 622 / 624.6Syn Z: -1.26

ClinVar Variant Classifications

403 submitted variants in ClinVar

Classification Summary

Pathogenic37
Likely Pathogenic11
VUS224
Likely Benign66
Benign38
Conflicting3
37
Pathogenic
11
Likely Pathogenic
224
VUS
66
Likely Benign
38
Benign
3
Conflicting

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
11
0
26
0
37
Likely Pathogenic
7
0
4
0
11
VUS
3
202
17
2
224
Likely Benign
0
15
23
28
66
Benign
0
13
15
10
38
Conflicting
3
Total212308540379

LoF = frameshift, stop gained/lost, canonical splice · Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

TBC1D32 · 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
Open Research Assistant →
Key Publications
Landmark & review papers · by relevance
PubMed
Top 5 results · since 2015Search PubMed ↗
Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC