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GeneE
10 sources retrieved Β· Most recent: April 2026 Β· Index updated 15 days ago
β“˜GeneE is for informational purposes only. It is not a substitute for professional medical advice, diagnosis, or treatment.
ABCA12
ATP binding cassette subfamily A member 12
Chromosome 2 Β· 2q35
NCBI Gene: 26154Ensembl: ENSG00000144452.15HGNC: HGNC:14637UniProt: B3KVV3
62PubMed Papers
22Diseases
0Drugs
207Pathogenic Variants
FUNCTIONAL ROLE
Transporter
RESEARCH IMPACT
Variant-Rich
CLINICAL
OMIM Disease Gene
DATA QUALITY
βœ“ Experimental GO Evidenceβœ“ Swiss-Prot Reviewed
phospholipid effluxregulated exocytosisceramide transportprotein bindingautosomal recessive congenital ichthyosis 4Aautosomal recessive congenital ichthyosis 4BHarlequin ichthyosislamellar ichthyosis
✦AI Summary

ABCA12 encodes an ATP-binding cassette transporter that plays critical roles in skin barrier function and lipid homeostasis. The protein transports lipids, particularly glucosylceramides, from the outer to inner leaflet of lamellar granule membranes in keratinocytes, facilitating lipid lamellae formation in the stratum corneum essential for epidermal barrier function 1. ABCA12 also regulates keratinocyte differentiation and desquamation by transporting lamellar granule-associated proteolytic enzymes 1. The upper hair follicle forms a functioning barrier through ABCA12, and barrier disruption in this region affects epidermal responses and impairs sebum release 1. Loss-of-function mutations in ABCA12 cause autosomal recessive congenital ichthyosis, with biallelic loss-of-function mutations typically resulting in the severe harlequin ichthyosis phenotype, while missense mutations generally lead to milder forms like congenital ichthyosiform erythroderma or lamellar ichthyosis 2. Beyond dermatological functions, ABCA12 has been implicated in cancer stem cell biology, where it maintains stemness and chemoresistance by reducing intracellular ceramide levels 3, and recent studies suggest potential roles in autism neurobiology through effects on lipid homeostasis and neuroinflammation 4.

Sources cited
1
ABCA12 transports lipids in keratinocytes, forms barrier function in upper hair follicle, and regulates desquamation
PMID: 38941190
2
Loss-of-function mutations cause harlequin ichthyosis while missense mutations cause milder ichthyosis forms
PMID: 36980989
3
ABCA12 maintains cancer stem cell function by reducing intracellular ceramide levels
PMID: 38039374
4
ABCA12 has emerging roles in autism neurobiology through lipid homeostasis and neuroinflammation
PMID: 40366508
⚠Limited data available β€” This gene has 4 indexed publications. Summary and analysis may be incomplete.
Disease Associationsβ“˜22
autosomal recessive congenital ichthyosis 4AOpen Targets
0.80Strong
autosomal recessive congenital ichthyosis 4BOpen Targets
0.79Strong
Harlequin ichthyosisOpen Targets
0.75Strong
lamellar ichthyosisOpen Targets
0.64Moderate
autosomal recessive congenital ichthyosisOpen Targets
0.48Moderate
congenital reticular ichthyosiform erythrodermaOpen Targets
0.41Moderate
congenital non-bullous ichthyosiform erythrodermaOpen Targets
0.39Weak
ichthyosisOpen Targets
0.37Weak
neurodegenerative diseaseOpen Targets
0.36Weak
placenta praeviaOpen Targets
0.28Weak
exostosisOpen Targets
0.27Weak
multinodular goiterOpen Targets
0.27Weak
Abnormality of the skinOpen Targets
0.27Weak
bilirubin metabolism diseaseOpen Targets
0.24Weak
cholelithiasisOpen Targets
0.23Weak
inherited ichthyosisOpen Targets
0.20Weak
genetic disorderOpen Targets
0.19Weak
carotid artery diseaseOpen Targets
0.16Weak
neoplasmOpen Targets
0.08Suggestive
esophageal squamous cell carcinomaOpen Targets
0.07Suggestive
Ichthyosis, congenital, autosomal recessive 4AUniProt
Ichthyosis, congenital, autosomal recessive 4BUniProt
Pathogenic Variants207
NM_173076.3(ABCA12):c.4139A>G (p.Asn1380Ser)Pathogenic
Autosomal recessive congenital ichthyosis 4A|not provided|Autosomal recessive congenital ichthyosis 4B;Autosomal recessive congenital ichthyosis 4A
β˜…β˜…β˜†β˜†2025β†’ Residue 1380
NM_173076.3(ABCA12):c.2140C>T (p.Arg714Ter)Pathogenic
not provided|Autosomal recessive congenital ichthyosis 4A;Autosomal recessive congenital ichthyosis 4B
β˜…β˜…β˜†β˜†2025β†’ Residue 714
NM_173076.3(ABCA12):c.6858del (p.Phe2286fs)Pathogenic
not provided|Autosomal recessive congenital ichthyosis 4A;Autosomal recessive congenital ichthyosis 4B
β˜…β˜…β˜†β˜†2025β†’ Residue 2286
NM_173076.3(ABCA12):c.3381_3481del (p.Ile1128fs)Pathogenic
not provided|Ichthyosis and erythrokeratoderma
β˜…β˜…β˜†β˜†2025β†’ Residue 1128
NM_173076.3(ABCA12):c.3256A>T (p.Lys1086Ter)Pathogenic
not provided|ABCA12-related disorder
β˜…β˜…β˜†β˜†2025β†’ Residue 1086
NM_173076.3(ABCA12):c.3548A>G (p.Tyr1183Cys)Pathogenic
Lamellar ichthyosis|not provided
β˜…β˜…β˜†β˜†2025β†’ Residue 1183
NM_173076.3(ABCA12):c.196G>A (p.Gly66Arg)Likely pathogenic
not provided|Lamellar ichthyosis
β˜…β˜…β˜†β˜†2025β†’ Residue 66
NM_173076.3(ABCA12):c.179G>C (p.Arg60Pro)Pathogenic
not provided|Lamellar ichthyosis
β˜…β˜…β˜†β˜†2025β†’ Residue 60
NM_173076.3(ABCA12):c.1210C>T (p.Arg404Ter)Pathogenic
not provided|Lamellar ichthyosis
β˜…β˜…β˜†β˜†2025β†’ Residue 404
NM_173076.3(ABCA12):c.2785C>T (p.Arg929Cys)Likely pathogenic
Ichthyosis|Autosomal recessive congenital ichthyosis 4A;Autosomal recessive congenital ichthyosis 4B
β˜…β˜…β˜†β˜†2025β†’ Residue 929
NM_173076.3(ABCA12):c.7444C>T (p.Arg2482Ter)Pathogenic
not provided|Autosomal recessive congenital ichthyosis 4B|Lamellar ichthyosis
β˜…β˜…β˜†β˜†2025β†’ Residue 2482
NM_173076.3(ABCA12):c.3470C>T (p.Ser1157Leu)Pathogenic
not provided|Lamellar ichthyosis
β˜…β˜…β˜†β˜†2025β†’ Residue 1157
NM_173076.3(ABCA12):c.7277G>A (p.Arg2426Gln)Likely pathogenic
Autosomal recessive congenital ichthyosis 4B|not provided|Lamellar ichthyosis
β˜…β˜…β˜†β˜†2025β†’ Residue 2426
NM_173076.3(ABCA12):c.1300C>T (p.Arg434Ter)Pathogenic
not provided|Autosomal recessive congenital ichthyosis 4B;Autosomal recessive congenital ichthyosis 4A|Lamellar ichthyosis|Autosomal recessive congenital ichthyosis 4B
β˜…β˜…β˜†β˜†2025β†’ Residue 434
NM_173076.3(ABCA12):c.178C>T (p.Arg60Ter)Pathogenic
not provided
β˜…β˜…β˜†β˜†2024β†’ Residue 60
NM_173076.3(ABCA12):c.3882G>A (p.Trp1294Ter)Pathogenic
not provided|Autosomal recessive congenital ichthyosis 4A|Autosomal recessive congenital ichthyosis 4B|Autosomal recessive congenital ichthyosis 4B;Autosomal recessive congenital ichthyosis 4A
β˜…β˜…β˜†β˜†2024β†’ Residue 1294
NM_173076.3(ABCA12):c.4540C>T (p.Arg1514Cys)Pathogenic
Autosomal recessive congenital ichthyosis 4A|not provided|Lamellar ichthyosis
β˜…β˜…β˜†β˜†2024β†’ Residue 1514
NM_173076.3(ABCA12):c.6610C>T (p.Arg2204Ter)Pathogenic
Autosomal recessive congenital ichthyosis 4B|not provided|Autosomal recessive congenital ichthyosis 4A|Lamellar ichthyosis
β˜…β˜…β˜†β˜†2024β†’ Residue 2204
NM_173076.3(ABCA12):c.2956C>T (p.Arg986Trp)Pathogenic
not provided|Lamellar ichthyosis
β˜…β˜…β˜†β˜†2024β†’ Residue 986
NM_173076.3(ABCA12):c.4541G>A (p.Arg1514His)Pathogenic
Autosomal recessive congenital ichthyosis 4A|ABCA12-related disorder|Lamellar ichthyosis
β˜…β˜…β˜†β˜†2024β†’ Residue 1514
View on ClinVar β†—
Related Genes
NIPAL4Protein interaction96%ALOX12BProtein interaction95%TGM1Protein interaction95%CYP4F22Protein interaction92%ALOXE3Protein interaction90%LIPNProtein interaction87%
Tissue Expression6 tissues
Brain
100%
Ovary
84%
Lung
26%
Liver
19%
Bone Marrow
6%
Heart
0%
Gene Interaction Network
Click a node to explore
ABCA12NIPAL4ALOX12BTGM1CYP4F22ALOXE3LIPN
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted Β· UniProt Q86UK0
View on AlphaFold β†—
Constraintβ“˜
LOEUFβ“˜
0.58Moderately Constrained
pLIβ“˜
0.00Tolerant
Observed/Expected LoF0.51 [0.44–0.58]
RankingsWhere ABCA12 stands among ~20K protein-coding genes
  • #7,425of 20,598
    Most Researched62
  • #321of 5,498
    Most Pathogenic Variants207 Β· top 10%
  • #3,902of 17,882
    Most Constrained (LOEUF)0.58 Β· top quartile
Genes detectedABCA12
Sources retrieved10 papers
Response timeβ€”
πŸ“„ Sources
10β–Ό
1
Keratosis pilaris: an update and approach to management.
PMID: 37166753
Ital J Dermatol Venerol Β· 2023
1.00
2
Meta-Analysis of Mutations in
PMID: 33435499
Genes (Basel) Β· 2021
0.90
3
Clinical and Genetic Findings in Patients With Palmoplantar Keratoderma.
PMID: 39630431
JAMA Dermatol Β· 2025
0.80
4
Targeting ABCA12-controlled ceramide homeostasis inhibits breast cancer stem cell function and chemoresistance.
PMID: 38039374
Sci Adv Β· 2023
0.70
5
From Genetics to Function: the Role of ABCA12 in Autism Neurobiology.
PMID: 40366508
J Mol Neurosci Β· 2025
0.60