KICS2

KICSTOR subunit 2, formerly C12orf66

Synonyms:: KICS2, C12ORF66, FLJ32549

Phenotypes

Human ciliopathy phenotype:: Bi-allelic variants in KICS2 cause intellectual disability and epilepsy (11 affected individuals).

Ciliopathy associations

Intellectual Developmental Disorder with Epilepsy

Subcellular localization

Ciliary associated gene, Lysosomes, Cytokinetic bridge

Functional category

Motile cilium & axoneme
Ciliary assembly/disassembly
Cilia–cytoskeleton/adhesion links

Function

Component of KICSTOR complex regulating mTORC1 in response to nutrient deprivation. Variants compromise KICSTOR complex formation and impact mTORC1 proteome including ciliogenesis-related proteins. Kics2 depletion in zebrafish causes ciliary dysfunction (Buchert et al. 2025, PMID 39824192).

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