SPG21

Chr 15

SPG21 abhydrolase domain containing, maspardin

Also known as: ABHD21, ACP33, BM-019, GL010, MAST

The protein encoded by this gene binds to the hydrophobic C-terminal amino acids of CD4 which are involved in repression of T cell activation. The interaction with CD4 is mediated by the noncatalytic alpha/beta hydrolase fold domain of this protein. It is thus proposed that this gene product modulates the stimulatory activity of CD4. Mutations in this gene are associated with autosomal recessive spastic paraplegia 21 (SPG21), also known as mast syndrome. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Aug 2014]

GeneReviewsResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtSpastic paraplegia 21, autosomal recessive
0
Active trials
4
Pubs (1 yr)
P/LP submissions
P/LP missense
0.83
LOEUF
Mechanism
📖
GeneReview available — SPG21
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?
0.83LOEUF
pLI 0.001
Z-score 2.07
OE 0.46 (0.270.83)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?
1.16Z-score
OE missense 0.75 (0.650.87)
127 obs / 169.4 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.46 (0.270.83)
00.351.4
Missense OE?0.75 (0.650.87)
00.61.4
Synonymous OE?1.08
01.21.6
LoF obs/exp: 8 / 17.3Missense obs/exp: 127 / 169.4Syn Z: -0.51

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

SPG21 · 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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