SDHAF1

Chr 19AR

succinate dehydrogenase complex assembly factor 1

Also known as: LYRM8, MC2DN2

The succinate dehydrogenase (SDH) complex (or complex II) of the mitochondrial respiratory chain is composed of 4 individual subunits. The protein encoded by this gene resides in the mitochondria, and is essential for SDH assembly, but does not physically associate with the complex in vivo. Mutations in this gene are associated with SDH-defective infantile leukoencephalopathy (mitochondrial complex II deficiency).[provided by RefSeq, Mar 2010]

OMIMResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

Mitochondrial complex II deficiency, nuclear type 2MIM #619166
AR

Clinical highlights

Gene-disease validity (ClinGen)
Leigh syndrome · ARLimitednot for standalone diagnostic reporting2 gene-disease associations curated in total
0
Active trials
1
Pubs (1 yr)
P/LP submissions
P/LP missense
1.90
LOEUF
Multiple*
Mechanism· predicted

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
1.90LOEUF
pLI 0.087
Z-score -0.09
OE 1.11 (0.281.90)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
0.38Z-score
OE missense 0.86 (0.681.09)
49 obs / 57.1 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?1.11 (0.281.90)
00.351.4
Missense OE?0.86 (0.681.09)
00.61.4
Synonymous OE?0.77
01.21.6
LoF obs/exp: 1 / 0.9Missense obs/exp: 49 / 57.1Syn Z: 0.94

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

SDHAF1 · 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 →