AP4M1

Chr 7

adaptor related protein complex 4 subunit mu 1

Also known as: CPSQ3, MU-4, MU-ARP2, SPG50

This gene encodes a subunit of the heterotetrameric AP-4 complex. The encoded protein belongs to the adaptor complexes medium subunits family. This AP-4 complex is involved in the recognition and sorting of cargo proteins with tyrosine-based motifs from the trans-golgi network to the endosomal-lysosomal system. [provided by RefSeq, Jul 2008]

GeneReviewsResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtSpastic paraplegia 50, autosomal recessive

Clinical highlights

Gene-disease validity (ClinGen)
AP-4 deficiency syndrome · ARDefinitivesufficient evidence for diagnostic panels
Interpreting a novel variant
Loss of function is the curated mechanism (Gene2Phenotype), though the gene is not strongly LoF-constrained in the population — weigh truncating variants against that tolerance.Curated gene-level mechanism — a prior for triage, not a per-variant call.
4
Active trials
13
Pubs (1 yr)
P/LP submissions
P/LP missense
1.31
LOEUF
LOF
Mechanism· G2P
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GeneReview available — AP4M1
Authoritative clinical overview · Recommended first read
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Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
1.31LOEUF
pLI 0.000
Z-score 0.26
OE 0.95 (0.691.31)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
-1.21Z-score
OE missense 1.21 (1.101.33)
311 obs / 256.4 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios?
LoF OE?0.95 (0.691.31)
00.351.4
Missense OE?1.21 (1.101.33)
00.61.4
Synonymous OE?1.39
01.21.6
LoF obs/exp: 26 / 27.5Missense obs/exp: 311 / 256.4Syn Z: -3.23

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

AP4M1 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.