SPART

Chr 13

spartin

Also known as: SPG20, TAHCCP1

This gene encodes a protein containing a MIT (Microtubule Interacting and Trafficking molecule) domain, and is implicated in regulating endosomal trafficking and mitochondria function. The protein localizes to mitochondria and partially co-localizes with microtubules. Stimulation with epidermal growth factor (EGF) results in protein translocation to the plasma membrane, and the protein functions in the degradation and intracellular trafficking of EGF receptor. Multiple alternatively spliced variants, encoding the same protein, have been identified. Mutations associated with this gene cause autosomal recessive spastic paraplegia 20 (Troyer syndrome). [provided by RefSeq, Nov 2008]

GeneReviewsResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtSpastic paraplegia 20, autosomal recessive

Clinical highlights

Gene-disease validity (ClinGen)
Troyer syndrome · ARDefinitivesufficient evidence for diagnostic panels
0
Active trials
17
Pubs (1 yr)
P/LP submissions
P/LP missense
0.84
LOEUF
GOF
Mechanism· predicted
📖
GeneReview available — SPART
Authoritative clinical overview · Recommended first read
Open GeneReview ↗
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?
0.84LOEUF
pLI 0.000
Z-score 2.22
OE 0.54 (0.360.84)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?
0.07Z-score
OE missense 0.99 (0.911.08)
346 obs / 349.7 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.54 (0.360.84)
00.351.4
Missense OE?0.99 (0.911.08)
00.61.4
Synonymous OE?1.00
01.21.6
LoF obs/exp: 15 / 27.5Missense obs/exp: 346 / 349.7Syn Z: 0.01

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

SPART · 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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