PAMR1

Chr 11

peptidase domain containing associated with muscle regeneration 1

Also known as: DKFZP586H2123, FP938, RAMP

The PAMR1 protein is predicted to function as a calcium-binding serine protease involved in proteolysis and may contribute to skeletal muscle regeneration. Mutations cause autosomal recessive myopathy with early childhood onset. The gene shows tolerance to loss-of-function variants, consistent with a recessive inheritance pattern.

Summary from RefSeq, UniProt
Research Assistant →
1
Active trials
8
Pubs (1 yr)
15
P/LP submissions
0%
P/LP missense
1.43
LOEUF
Mechanism
Clinical SummaryPAMR1
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
15 unique Pathogenic / Likely Pathogenic· 107 VUS of 137 total submissions
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Clinical Trials
1 active or recruiting trial — potential therapeutic options may be available

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.43LOEUF
pLI 0.000
Z-score -0.68
OE 1.11 (0.881.43)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-0.46Z-score
OE missense 1.06 (0.981.15)
460 obs / 432.8 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE1.11 (0.881.43)
00.351.4
Missense OE1.06 (0.981.15)
00.61.4
Synonymous OE1.04
01.21.6
LoF obs/exp: 45 / 40.4Missense obs/exp: 460 / 432.8Syn Z: -0.38

ClinVar Variant Classifications

137 submitted variants in ClinVar

Classification Summary

Pathogenic15
VUS107
Likely Benign5
15
Pathogenic
107
VUS
5
Likely Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
15
0
15
Likely Pathogenic
0
0
0
0
0
VUS
0
97
10
0
107
Likely Benign
0
4
0
1
5
Benign
0
0
0
0
0
Total0101251127

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

View in ClinVar →

Protein Context — Lollipop Plot

PAMR1 · protein map & ClinVar variants

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

Clinical Literature
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