SPG7

Chr 16

SPG7 matrix AAA peptidase subunit, paraplegin

Also known as: CAR, CMAR, PGN, SPG5C

This gene encodes a mitochondrial metalloprotease protein that is a member of the AAA family. Members of this protein family share an ATPase domain and have roles in diverse cellular processes including membrane trafficking, intracellular motility, organelle biogenesis, protein folding, and proteolysis. Mutations in this gene cause autosomal recessive spastic paraplegia 7. Two transcript variants encoding distinct isoforms have been identified. [provided by RefSeq, Mar 2014]

GeneReviewsResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtSpastic paraplegia 7, autosomal recessive, with or without cerebellar ataxia

Clinical highlights

Gene-disease validity (ClinGen)
mitochondrial disease · ARDefinitivesufficient evidence for diagnostic panels2 gene-disease associations curated in total
0
Active trials
52
Pubs (1 yr)
P/LP submissions
P/LP missense
1.65
LOEUF
DN
Mechanism· predicted
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GeneReview available — SPG7
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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?
1.65LOEUF
pLI 0.000
Z-score -1.69
OE 1.30 (1.031.65)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
-0.87Z-score
OE missense 1.11 (1.031.20)
528 obs / 474.5 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios?
LoF OE?1.30 (1.031.65)
00.351.4
Missense OE?1.11 (1.031.20)
00.61.4
Synonymous OE?1.38
01.21.6
LoF obs/exp: 48 / 36.9Missense obs/exp: 528 / 474.5Syn Z: -4.22

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

SPG7 · protein map & ClinVar variants

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

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

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