SPTA1

Chr 1

spectrin alpha, erythrocytic 1

Also known as: EL2, HPP, HS3, SPH3, SPTA

This gene encodes a member of a family of molecular scaffold proteins that link the plasma membrane to the actin cytoskeleton and functions in the determination of cell shape, arrangement of transmembrane proteins, and organization of organelles. The encoded protein is primarily composed of 22 spectrin repeats which are involved in dimer formation. It forms a component of the erythrocyte plasma membrane. Mutations in this gene result in a variety of hereditary red blood cell disorders, including elliptocytosis-2, pyropoikilocytosis, and spherocytosis, type 3. [provided by RefSeq, Aug 2017]

ResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtElliptocytosis 2
UniProtHereditary pyropoikilocytosis
UniProtSpherocytosis 3
0
Active trials
38
Pubs (1 yr)
P/LP submissions
P/LP missense
0.49
LOEUF
Multiple*
Mechanism· predicted
Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint?
0.49LOEUF
pLI 0.000
Z-score 6.75
OE 0.39 (0.320.49)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint?
-2.25Z-score
OE missense 1.18 (1.131.23)
1456 obs / 1233.7 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios?
LoF OE?0.39 (0.320.49)
00.351.4
Missense OE?1.18 (1.131.23)
00.61.4
Synonymous OE?1.17
01.21.6
LoF obs/exp: 57 / 144.6Missense obs/exp: 1456 / 1233.7Syn Z: -2.89

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

SPTA1 · 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.

Search ClinicalTrials.gov →