CARHSP1

Chr 16

calcium regulated heat stable protein 1

Also known as: CRHSP-24, CRHSP24, CSDC1

The protein binds mRNA and regulates mRNA stability, with predicted roles in mRNA 3'-UTR binding and regulation of mRNA stability in the cytoplasm. Mutations cause autosomal recessive spastic paraplegia with intellectual disability, typically presenting in early childhood with progressive spasticity and developmental delays. The gene shows low constraint against loss-of-function variants, consistent with its autosomal recessive inheritance pattern.

OMIMResearchSummary from RefSeq, UniProt
GOFmechanismLOEUF 1.87
Clinical SummaryCARHSP1
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
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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
1.87LOEUF
pLI 0.000
Z-score -0.35
OE 1.17 (0.621.87)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-2.38Z-score
OE missense 1.69 (1.481.91)
160 obs / 94.8 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE1.17 (0.621.87)
00.351.4
Missense OE1.69 (1.481.91)
00.61.4
Synonymous OE2.21
01.21.6
LoF obs/exp: 6 / 5.1Missense obs/exp: 160 / 94.8Syn Z: -5.94
DN
0.5771th %ile
GOF
0.6540th %ile
LOF
0.56top 25%

The highest-scoring mechanism for this gene is gain-of-function.

GOFprediction above median

Note: In-silico variant effect predictors (SIFT, PolyPhen, REVEL, CADD) may underestimate pathogenicity of missense variants in genes with GOF or DN mechanisms. Consider functional evidence and clinical context.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

CARHSP1 · protein map & ClinVar variants

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

3D Protein StructureAlphaFold
Clinical Literature
Open Research Assistant →
Key Publications
Landmark & review papers · by relevance
PubMed
Top 1 results · since 2015Search PubMed ↗