LRSAM1

Chr 9

leucine rich repeat and sterile alpha motif containing 1

Also known as: CMT2P, RIFLE, TAL

This gene encodes a ring finger protein involved in a variety of functions, including regulation of signaling pathways and cell adhesion, mediation of self-ubiquitylation, and involvement in cargo sorting during receptor endocytosis. Mutations in this gene have been associated with Charcot-Marie-Tooth disease. Multiple transcript variants encoding different isoforms have been identified for this gene. [provided by RefSeq, Jan 2012]

GeneReviewsResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtCharcot-Marie-Tooth disease, axonal, type 2P

Clinical highlights

Gene-disease validity (ClinGen)
Charcot-Marie-Tooth disease axonal type 2P · ADDefinitivesufficient evidence for diagnostic panels
0
Active trials
12
Pubs (1 yr)
P/LP submissions
P/LP missense
0.75
LOEUF
Multiple*
Mechanism· predicted
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GeneReview available — LRSAM1
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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?
0.75LOEUF
pLI 0.000
Z-score 3.00
OE 0.55 (0.410.75)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?
0.41Z-score
OE missense 0.94 (0.871.03)
399 obs / 422.6 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.55 (0.410.75)
00.351.4
Missense OE?0.94 (0.871.03)
00.61.4
Synonymous OE?1.05
01.21.6
LoF obs/exp: 29 / 52.4Missense obs/exp: 399 / 422.6Syn Z: -0.48

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

LRSAM1 · 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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