CWH43
cell wall biogenesis 43 C-terminal homolog
- Ensembl:
- ENSG00000109182
- UniProt:
- Q9H720
- OMIM:
- 618561
- Synonyms:
- CWH43-C, FLJ21511, PGAP2IP
Cilia effects upon perturbation of CWH43
- Cilia number / % ciliated:
- Decreased cilia number
Ciliogenesis screen results (3 screens)
- Wheway et al. 2015 (siRNA) [siRNA]: Ciliogenesis Defect (z=-2.78) PMID:26167766
- Breslow et al. 2018 (CRISPR) [CRISPR]: No Significant Effect PMID:29459680
- Basu et al. 2023 (siRNA) [siRNA]: Left-Right Asymmetry Candidate (Z2MCilia=0.91) PMID:37771269
Phenotypes
- Mouse phenotype:
- abnormal locomotor behavior
Subcellular localization
cilia
Functional category
- Ciliary assembly/disassembly
- Actin & cytoskeleton regulation
- Protein processing & maturation
Function
Six of the iNPH-associated proteins examined (Cwh43, Rxfp2, Ak9, HAVCR1/Kim1, spatacsin, and myo7A) localized to ependymal motile cilia in vivo or in cultured mouse ependymal cells (38100419). Decreased numbers of ependymal cilia.
Model organism evidence
Mice homozygous for an iNPH-associated AK9 mutation displayed normal cilia structure and number, but decreased cilia motility and beat frequency, communicating hydrocephalus, and balance impairment.
Mice heterozygous for CWH43 deletion appeared grossly normal but displayed hydrocephalus, gait and balance abnormalities, decreased numbers of ependymal cilia, and decreased localization of glycosylphosphatidylinositol-anchored proteins to the apical surfaces of choroid plexus and ependymal cell