Recombinant Human Succinate dehydrogenase assembly factor 1, mitochondrial (SDHAF1)

Uniprot Id

A6NFY7

Target Species

Human

Target Name

SDHAF1

Target Full Name

Succinate dehydrogenase assembly factor 1, mitochondrial

Target Function

Plays an essential role in the assembly of succinate dehydrogenase (SDH), an enzyme complex (also referred to as respiratory complex II) that is a component of both the tricarboxylic acid (TCA) cycle and the mitochondrial electron transport chain, and which couples the oxidation of succinate to fumarate with the reduction of ubiquinone (coenzyme Q) to ubiquinol. Promotes maturation of the iron-sulfur protein subunit SDHB of the SDH catalytic dimer, protecting it from the deleterious effects of oxidants. May act together with SDHAF3. Contributes to iron-sulfur cluster incorporation into SDHB by binding to SDHB and recruiting the iron-sulfur transfer complex formed by HSC20, HSPA9 and ISCU through direct binding to HSC20.

Target Involvement

Mitochondrial complex II deficiency (MT-C2D)

Target Subcellular Location

Mitochondrion matrix.

Target Protein Families

Complex I LYR family, SDHAF1 subfamily

Target Tissue Specificity

Ubiquitously expressed.

Target Synonyms

LYR motif containing 8; LYR motif-containing protein 8; LYRM8; mitochondrial; SDH assembly factor 1; SDHAF1; SDHF1_HUMAN; Succinate dehydrogenase assembly factor 1; Succinate dehydrogenase assembly factor 1 mitochondrial; Succinate dehydrogenase complex assembly factor 1