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GeneE
7 sources retrieved Β· Most recent: April 2026 Β· Index updated 15 days ago
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CFAP54
cilia and flagella associated protein 54
Chromosome 12 Β· 12q23.1
NCBI Gene: 144535Ensembl: ENSG00000188596.11HGNC: HGNC:26456UniProt: Q96N23
18PubMed Papers
22Diseases
0Drugs
7Pathogenic Variants
CLINICAL
OMIM Disease Gene
DATA QUALITY
βœ“ Experimental GO Evidenceβœ“ Swiss-Prot Reviewed
sperm flagellum assemblyspermatogenesiscilium assemblycilium movement involved in cell motilityspermatogenic failure 98ciliary dyskinesia, primary, 54cervical carcinomahypersomnia
✦AI Summary

CFAP54 (cilia and flagella associated protein 54) is essential for assembly and function of motile cilia and flagella, functioning as a component of the C1d projection of the central microtubule apparatus 1. The protein is required for proper axoneme organization and ciliary movement involved in cell motility [GO annotations]. In spermatogenesis, CFAP54 is critical for sperm flagellum assembly and male fertility, with deficiency resulting in abnormal sperm morphology, reduced motility and concentration, and axoneme disorganization 2. Pathogenic variants in CFAP54 cause two distinct clinical phenotypes. In primary ciliary dyskinesia (PCD), CFAP54 mutations lead to defective mucociliary clearance with characteristic features including hydrocephalus, chr12 airway infections, and nasal mucus accumulation, despite normal nasal nitric oxide levels and ciliary beating patterns 13. In male infertility, biallelic CFAP54 mutations cause spermatogenic failure with multiple morphological abnormalities of sperm flagella (MMAF) and non-obstructive azoospermia (NOA), accompanied by reduced expression of flagellar assembly proteins including IFT20, IFT52, IFT122, and SPEF2 245. Notably, intracytoplasmic sperm injection can achieve successful pregnancies despite CFAP54 mutations 2. CFAP54 represents an important genetic diagnostic target for both PCD and male infertility.

Sources cited
1
CFAP54 is a C1d projection component of the central microtubule apparatus; mutations cause PCD with hydrocephalus, infertility, and nasal mucus accumulation
PMID: 37725231
2
Biallelic CFAP54 mutations cause severe MMAF and NOA with axoneme disorganization and reduced flagellar assembly protein expression
PMID: 36593121
3
CFAP54 is a novel PCD gene associated with C1d-defective primary ciliary dyskinesia that evades standard diagnostic algorithms
PMID: 39362668
4
CFAP54 variants identified as causative genes in male infertility cohort; involved in spermatogenesis
PMID: 39267058
5
CFAP54 identified as a high-risk gene for NOA with high/specific testicular expression
PMID: 36259570
Disease Associationsβ“˜22
spermatogenic failure 98Open Targets
0.49Moderate
ciliary dyskinesia, primary, 54Open Targets
0.46Moderate
cervical carcinomaOpen Targets
0.41Moderate
hypersomniaOpen Targets
0.27Weak
crush injuryOpen Targets
0.27Weak
response to antihypertensive drugOpen Targets
0.27Weak
poisoningOpen Targets
0.26Weak
respiratory system diseaseOpen Targets
0.24Weak
polyarteritis nodosaOpen Targets
0.21Weak
adolescent idiopathic scoliosisOpen Targets
0.10Suggestive
azoospermiaOpen Targets
0.08Suggestive
primary ciliary dyskinesiaOpen Targets
0.07Suggestive
partial chromosome Y deletionOpen Targets
0.05Suggestive
spermatogenic failure 65Open Targets
0.05Suggestive
spermatogenic failure 54Open Targets
0.05Suggestive
spermatogenic failure 84Open Targets
0.05Suggestive
spermatogenic failure 93Open Targets
0.05Suggestive
spermatogenic failure, X-linked, 3Open Targets
0.05Suggestive
spermatogenic failure 56Open Targets
0.05Suggestive
spermatogenic failure 92Open Targets
0.05Suggestive
Ciliary dyskinesia, primary, 54UniProt
Spermatogenic failure 98UniProt
Pathogenic Variants7
NM_001306084.2(CFAP54):c.8644_8645del (p.Leu2882fs)Likely pathogenic
Spermatogenic failure 98;Ciliary dyskinesia, primary, 54
β˜…β˜†β˜†β˜†2025β†’ Residue 2882
NM_001306084.2(CFAP54):c.4713_4716del (p.Phe1571fs)Likely pathogenic
not provided
β˜…β˜†β˜†β˜†2025β†’ Residue 1571
NM_001306084.2(CFAP54):c.955C>T (p.Arg319Cys)Pathogenic
Spermatogenic failure 98
β˜†β˜†β˜†β˜†2025β†’ Residue 319
NM_001306084.2(CFAP54):c.937G>A (p.Gly313Arg)Pathogenic
Spermatogenic failure 98
β˜†β˜†β˜†β˜†2025β†’ Residue 313
NM_001306084.2(CFAP54):c.4885C>T (p.Arg1629Cys)Pathogenic
Spermatogenic failure 98
β˜†β˜†β˜†β˜†2025β†’ Residue 1629
NM_001306084.2(CFAP54):c.878G>A (p.Arg293His)Pathogenic
Spermatogenic failure 98
β˜†β˜†β˜†β˜†2025β†’ Residue 293
NM_001306084.2(CFAP54):c.3317del (p.Phe1106fs)Pathogenic
Spermatogenic failure 98
β˜†β˜†β˜†β˜†2025β†’ Residue 1106
View on ClinVar β†—
Related Genes
TMEM212Protein interaction78%WDPCPProtein interaction78%SPEF2Protein interaction74%CFAP221Protein interaction59%LRRC46Shared pathway50%DZIP1Shared pathway43%
Tissue Expression6 tissues
Brain
100%
Ovary
72%
Lung
72%
Liver
65%
Bone Marrow
24%
Heart
11%
Gene Interaction Network
Click a node to explore
CFAP54TMEM212WDPCPSPEF2CFAP221LRRC46DZIP1
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted Β· UniProt Q96N23
View on AlphaFold β†—
Constraintβ“˜
LOEUFβ“˜
0.93LoF Tolerant
pLIβ“˜
0.00Tolerant
Observed/Expected LoF0.82 [0.72–0.93]
RankingsWhere CFAP54 stands among ~20K protein-coding genes
  • #14,667of 20,598
    Most Researched18
  • #3,270of 5,498
    Most Pathogenic Variants7
  • #8,565of 17,882
    Most Constrained (LOEUF)0.93
Genes detectedCFAP54
Sources retrieved7 papers
Response timeβ€”
πŸ“„ Sources
7β–Ό
1
Whole exome sequencing analysis of 167 men with primary infertility.
PMID: 39267058
BMC Med Genomics Β· 2024
1.00
2
Pathogenic variants in
PMID: 39362668
Eur Respir J Β· 2024
0.86
3
Lack of CFAP54 causes primary ciliary dyskinesia in a mouse model and human patients.
PMID: 37725231
Front Med Β· 2023
0.71
4
Biallelic mutations in
PMID: 36593121
J Med Genet Β· 2023
0.57
5
Identification of risk genes in Chinese nonobstructive azoospermia patients based on whole-exome sequencing.
PMID: 36259570
Asian J Androl Β· 2023
0.43