TRAPPC2L

Chr 16AR

trafficking protein particle complex subunit 2L

Also known as: HSPC176, PERRB

This gene encodes a protein that interacts with the TRAPP complex to mediate vesicular transport from the endoplasmic reticulum to Golgi apparatus. Mutations cause progressive early-onset encephalopathy with episodic rhabdomyolysis, inherited in an autosomal recessive pattern. The gene is not highly constrained against loss-of-function variants in the general population.

OMIMResearchSummary from RefSeq, OMIM, UniProt
ARLOEUF 1.951 OMIM phenotype
Clinical SummaryTRAPPC2L
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
92 unique Pathogenic / Likely Pathogenic· 85 VUS of 272 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.95LOEUF
pLI 0.000
Z-score -1.69
OE 1.65 (1.021.95)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-0.38Z-score
OE missense 1.11 (0.951.31)
102 obs / 91.7 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE1.65 (1.021.95)
00.351.4
Missense OE1.11 (0.951.31)
00.61.4
Synonymous OE1.45
01.21.6
LoF obs/exp: 13 / 7.9Missense obs/exp: 102 / 91.7Syn Z: -2.20

ClinVar Variant Classifications

272 submitted variants in ClinVar

Classification Summary

Pathogenic76
Likely Pathogenic16
VUS85
Likely Benign57
Benign11
Conflicting9
76
Pathogenic
16
Likely Pathogenic
85
VUS
57
Likely Benign
11
Benign
9
Conflicting

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
9
4
63
0
76
Likely Pathogenic
4
5
7
0
16
VUS
0
60
24
1
85
Likely Benign
0
4
20
33
57
Benign
0
4
5
2
11
Conflicting
9
Total137711936254

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

View in ClinVar →

Protein Context — Lollipop Plot

TRAPPC2L · 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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