RAB33B

Chr 4AR

RAB33B, member RAS oncogene family

This gene encodes a small GTP-binding protein of the Rab GTPase family, whose members function in vesicle transport during protein secretion and endocytosis. Rab GTPases are active, membrane-associated proteins that recruit effector proteins in the GTP-bound state and inactive cytosolic proteins when in a GDP-bound state. The protein encoded by this gene is ubiquitously expressed and has been implicated in Golgi to endoplasmic reticulum cycling of Golgi enzymes. In addition, this protein regulates Golgi homeostasis and coordinates intra-Golgi retrograde trafficking. Allelic variants in this gene have been associated with Dyggve-Melchior-Clausen syndrome and Smith-McCort dysplasia 2, which are autosomal recessive spondyloepimetaphyseal dysplasias characterized by skeletal abnormalities. [provided by RefSeq, Sep 2016]

OMIMResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

Smith-McCort dysplasia 2MIM #615222
AR

Clinical highlights

Interpreting a novel variant
This gene is strongly intolerant of loss-of-function variation in the population, so LoF variants warrant close attention. No curated mechanism annotation is available — see the mechanism card for the computational prediction and its caveats.Based on population constraint only.
0
Active trials
7
Pubs (1 yr)
P/LP submissions
P/LP missense
0.60
LOEUF
Multiple*
Mechanism· predicted

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint?
0.60LOEUF
pLI 0.675
Z-score 2.28
OE 0.13 (0.040.60)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint?
0.63Z-score
OE missense 0.85 (0.720.99)
112 obs / 132.4 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.13 (0.040.60)
00.351.4
Missense OE?0.85 (0.720.99)
00.61.4
Synonymous OE?1.18
01.21.6
LoF obs/exp: 1 / 8.0Missense obs/exp: 112 / 132.4Syn Z: -1.02

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

RAB33B · 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.

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