Search results for FDXR

Showing 9 results out of 9

×

Species

Types

Compartments

Reaction types

Search properties

Species

Types

Compartments

Reaction types

Search properties

Protein (1 results from a total of 1)

Identifier: R-HSA-2395499
Species: Homo sapiens
Compartment: mitochondrial matrix
Primary external reference: UniProt: FDXR: P22570

Reaction (3 results from a total of 3)

Identifier: R-HSA-2395517
Species: Homo sapiens
Compartment: mitochondrial matrix
Two electrons and one proton are transferred from NADPH to the FAD moiety of ferredoxin reductase. A proton from the medium is also taken up by ferredoxin reductase.
Identifier: R-HSA-2395512
Species: Homo sapiens
Compartment: mitochondrial matrix
Two electrons are transferred from reduced ferredoxin reductase (FDXR, adrenodoxin reductase) to two ferredoxin-1 or ferredoxin-1L (FDX1, FDX1L) molecules, each of which binds one electron. Two protons are released during the reaction.
Identifier: R-HSA-5580269
Species: Homo sapiens
Compartment: mitochondrial matrix
Cholesterol side-chain cleavage enzyme, mitochondrial (CYP11A1) normally catalyses the side-chain cleavage of cholesterol to form pregnenolone. CYP11A1 is the active unit of a complex comprising adrenodoxin reductase (FDXR), adrenodoxin (FDX, or adrenodoxin-like protein FDX1L) (Strushkevich et al. 2011). Defects in CYP11A1 can cause Adrenal insufficiency, congenital, with 46,XY sex reversal (AICSR; MIM:613743). This is a rare disorder that can present as acute adrenal insufficiency in infancy with elevated ACTH and plasma renin activity and low or absent adrenal steroids. Although milder forms can present, associated with partial loss of enzyme activity, the severest phenotype is associated with prematurity, complete underandrogenisation and severe, early-onset adrenal failure. CYP11A1 mutations causing complete loss of function are a 6bp insertion resulting in the insertion of glycine and aspartate codons, a 1bp deletion resulting in a premature stop at 288 and the missense mutation V415E (Tajima et al. 2001, Hiort et al. 2005, Kim et al. 2008, Sahakitrungruang et al. 2010).

Complex (4 results from a total of 4)

Identifier: R-HSA-2395508
Species: Homo sapiens
Compartment: mitochondrial matrix
Identifier: R-HSA-2395502
Species: Homo sapiens
Compartment: mitochondrial matrix
Identifier: R-HSA-5580259
Species: Homo sapiens
Compartment: mitochondrial matrix
Identifier: R-HSA-5580249
Species: Homo sapiens
Compartment: mitochondrial matrix

Pathway (1 results from a total of 1)

Identifier: R-HSA-2395516
Species: Homo sapiens
Compartment: mitochondrial matrix
NADPH, ferredoxin reductase (FDXR, Adrenodoxin reductase), and ferredoxins (FDX1, FDX1L) comprise a short electron transport chain that provides electrons for biosynthesis of iron-sulfur clusters and steroid hormones (Sheftel et al. 2010, Shi et al. 2012, reviewed in Grinberg et al. 2000, Lambeth et al. 1982).
Cite Us!