KIDINS220

Chr 2

kinase D interacting substrate 220

Also known as: ARMS, SINO, VENARG

This gene encodes a transmembrane protein that is preferentially expressed in the nervous system where it controls neuronal cell survival, differentiation into exons and dendrites, and synaptic plasticity. The encoded protein interacts with membrane receptors, cytosolic signaling components, and cytoskeletal proteins, serving as a scaffold that mediates crosstalk between the neurotrophin pathway and several other intracellular signaling pathways. Aberrant expression of this gene is associated with the onset of various neuropsychiatric disorders and neurodegenerative diseases, including Alzheimer's disease. Naturally occurring mutations in this gene are associated with a syndrome characterized by spastic paraplegia, intellectual disability, nystagmus and obesity. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Feb 2017]

ResearchGenerating clinical summary…
LOFmechanismLOEUF 0.35
Clinical SummaryKIDINS220
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Gene-Disease Validity (ClinGen)
spastic paraplegia, intellectual disability, nystagmus, and obesity · ADModerate

Moderate evidence — consider for supplementary testing

2 total gene-disease associations curated

Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.24) despite low pLI — interpret in context.
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Clinical Trials
1 active or recruiting trial — potential therapeutic options may be available
Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint?
0.35LOEUF
pLI 0.074
Z-score 6.41
OE 0.24 (0.170.35)
Highly constrained

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

Missense Constraint?
2.26Z-score
OE missense 0.80 (0.750.84)
780 obs / 979.1 exp
Mild constraint

Moderately missense-constrained (top ~2.5%)

Observed / Expected Ratios?
LoF OE?0.24 (0.170.35)
00.351.4
Missense OE?0.80 (0.750.84)
00.61.4
Synonymous OE?0.99
01.21.6
LoF obs/exp: 20 / 83.0Missense obs/exp: 780 / 979.1Syn Z: 0.22

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

KIDINS220 · protein map & ClinVar variants

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