KPNA3

Chr 13q14.2

karyopherin subunit alpha 3

Aliases:: SRP1gamma, SRP4, hSRP1, IPOA4
MANE:: ENST00000261667.8

OMIM: 601892 ↗NCBI: 3839 ↗Ensembl ↗

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Predicted protein structure

AlphaFold predicted structure

KPNA3 · O00505

v62025-08-01

Predicted Aligned Error matrix for KPNA3 — diagonal shows residue index, colour shows pairwise prediction confidence (lower is better) — Predicted Aligned Error

Mean pLDDT

86.5/ 100

Confident

521 residues

Confidence breakdown

Very high(≥ 90)75%
Confident(70–90)5%
Low(50–70)9%
Very low(< 50)10%

Open interactive 3D viewer

AlphaFold (Jumper et al., 2021) · CC BY 4.0

Clinical relevance (Genomics England PanelApp)

Diagnostic Grade (Green)

Childhood onset hereditary spastic paraplegia
MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown
DDG2P
MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted

Disease associations (Open Targets)

spastic paraplegia 88, autosomal dominant
0.50
influenza
0.46
dengue disease
0.46
Abnormality of the skeletal system
0.42
autosomal dominant pure spastic paraplegia
0.37
Autosomal dominant spastic paraplegia type 37
0.37
atrial fibrillation
0.26
Alzheimer disease
0.25
Parkinson disease
0.24
neurodegenerative disease
0.24

Score is the Open Targets composite evidence score (0-1). Higher = stronger gene-disease association.

Protein function (UniProt)

Importin subunit alpha-4

Functions in nuclear protein import as an adapter protein for nuclear receptor KPNB1. Binds specifically and directly to substrates containing either a simple or bipartite NLS motif. Docking of the importin/substrate complex to the nuclear pore complex (NPC) is mediated by KPNB1 through binding to nucleoporin FxFG repeats and the complex is subsequently translocated through the pore by an energy requiring, Ran-dependent mechanism. At the nucleoplasmic side of the NPC, Ran binds to importin-beta and the three components separate and importin-alpha and -beta are re-exported from the nucleus to the cytoplasm where GTP hydrolysis releases Ran from importin. The directionality of nuclear import is thought to be conferred by an asymmetric distribution of the GTP- and GDP-bound forms of Ran between the cytoplasm and nucleus. In vitro, mediates the nuclear import of human cytomegalovirus UL84 by recognizing a non-classical NLS. Recognizes NLSs of influenza A virus nucleoprotein probably through ARM repeats 7-9