SYNCRIP

Chr 6

synaptotagmin binding cytoplasmic RNA interacting protein

Also known as: GRY-RBP, GRYRBP, HNRNPQ, HNRPQ1, NSAP1, PP68, hnRNP-Q

The protein is a heterogeneous nuclear ribonucleoprotein that regulates mRNA processing, splicing, stability, and translation, and is a component of the survival of motor neurons (SMN) complex. Mutations cause autosomal dominant neurodevelopmental disorders with intellectual disability, developmental delay, and seizures. This gene is highly constrained against loss-of-function variants, indicating that such mutations are likely to be pathogenic.

OMIMResearchSummary from RefSeq, UniProt
LOFmechanismLOEUF 0.21
Clinical SummarySYNCRIP
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Gene-Disease Validity (ClinGen)
SYNCRIP-related neurodevelopmental disorder · ADDefinitive

Definitive — sufficient evidence for diagnostic panels

Population Constraint (gnomAD)
Highly constrained gene — heterozygous loss-of-function variants are very rare in the population (pLI 1.00). One damaged copy is likely sufficient to cause disease.
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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

Dual constrained — LoF & missense intolerant
LoF Constraint
0.21LOEUF
pLI 0.999
Z-score 4.75
OE 0.07 (0.030.21)
Highly constrained

Highly LoF-intolerant (top ~10% of genes)

Missense Constraint
3.54Z-score
OE missense 0.47 (0.410.53)
163 obs / 349.1 exp
Constrained

Highly missense-constrained (top ~0.1%)

Observed / Expected Ratios
LoF OE0.07 (0.030.21)
00.351.4
Missense OE0.47 (0.410.53)
00.61.4
Synonymous OE1.10
01.21.6
LoF obs/exp: 2 / 30.1Missense obs/exp: 163 / 349.1Syn Z: -0.89
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
limitedSYNCRIP-related developmental disorderLOFAD
DN
0.3793th %ile
GOF
0.3491th %ile
LOF
0.76top 10%

The highest-scoring mechanism for this gene is loss-of-function (haploinsufficiency).

LOFprediction above median · LOEUF 0.21

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312. Mechanism ranking also informed by gnomAD constraint, ClinVar, and ClinGen data.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

SYNCRIP · protein map & ClinVar variants

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

3D Protein StructureAlphaFold
Clinical Literature
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