VPS37A

Chr 8AR

VPS37A subunit of ESCRT-I

Also known as: HCRP1, PQBP2, SPG53

This gene belongs to the VPS37 family, and encodes a component of the ESCRT-I (endosomal sorting complex required for transport I) protein complex, required for the sorting of ubiquitinated transmembrane proteins into internal vesicles of multivesicular bodies. Expression of this gene is downregulated in hepatocellular carcinoma, and mutations in this gene are associated with autosomal recessive spastic paraplegia-53. A related pseudogene has been identified on chromosome 5. Alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Dec 2012]

OMIMResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

Spastic paraplegia 53, autosomal recessiveMIM #614898
AR

Clinical highlights

Gene-disease validity (ClinGen)
complex hereditary spastic paraplegia · ARLimitednot for standalone diagnostic reporting
0
Active trials
10
Pubs (1 yr)
P/LP submissions
P/LP missense
0.72
LOEUF
DN
Mechanism· predicted

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
0.72LOEUF
pLI 0.001
Z-score 2.53
OE 0.41 (0.250.72)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?
-0.88Z-score
OE missense 1.18 (1.061.31)
235 obs / 199.8 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios?
LoF OE?0.41 (0.250.72)
00.351.4
Missense OE?1.18 (1.061.31)
00.61.4
Synonymous OE?1.27
01.21.6
LoF obs/exp: 9 / 21.7Missense obs/exp: 235 / 199.8Syn Z: -1.82

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

VPS37A · 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 →