BLOC1S5

Chr 6

biogenesis of lysosomal organelles complex 1 subunit 5

Also known as: BLOS5, HPS11, MU, MUTED

This gene encodes a component of BLOC-1 (biogenesis of lysosome-related organelles complex 1). Components of this complex are involved in the biogenesis of organelles such as melanosomes and platelet-dense granules. A mouse model for Hermansky-Pudlak Syndrome is mutated in the murine version of this gene. Alternative splicing results in multiple transcript variants. Read-through transcription exists between this gene and the upstream EEF1E1 (eukaryotic translation elongation factor 1 epsilon 1) gene, as well as with the downstream TXNDC5 (thioredoxin domain containing 5) gene. [provided by RefSeq, Dec 2010]

GeneReviewsResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtHermansky-Pudlak syndrome 11

Clinical highlights

Gene-disease validity (ClinGen)
Hermansky-Pudlak syndrome 11 · ARDefinitivesufficient evidence for diagnostic panels
0
Active trials
4
Pubs (1 yr)
P/LP submissions
P/LP missense
1.81
LOEUF
Multiple*
Mechanism· predicted
📖
GeneReview available — BLOC1S5
Authoritative clinical overview · Recommended first read
Open GeneReview ↗
Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
1.81LOEUF
pLI 0.000
Z-score -0.57
OE 1.19 (0.761.81)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
-0.25Z-score
OE missense 1.07 (0.921.25)
109 obs / 101.9 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios?
LoF OE?1.19 (0.761.81)
00.351.4
Missense OE?1.07 (0.921.25)
00.61.4
Synonymous OE?1.06
01.21.6
LoF obs/exp: 12 / 10.1Missense obs/exp: 109 / 101.9Syn Z: -0.27

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

BLOC1S5 · protein map & ClinVar variants

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

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

Search ClinicalTrials.gov →