MitoSciences: Enzymes

All Enzymes

2,4 dienoyl-CoA reductase (DECR1)
DECR1 is an auxiliary enzyme of mitochondrial fatty acid beta oxidation.

Acetyl-CoA acetyltransferase (ACAT1)
ACAT1 is a mitochondrial enzyme involved in both amino acid degradation and in fatty acid beta oxidation.

Acetyl-CoA acyltransferase, peroxisomal (ACAA1)
ACAA1 is involved in peroxisomal fatty acid oxidation.

Acetyl-CoA acyltransferase, mitochondrial (ACAA2)
ACAA2 is a homotetrameric enzyme catalyzing the fatty acid oxidation reaction Acyl CoA + acetyl CoA = CoA + 3-oxoacyl-CoA within the mitochondrion.

Aconitase (ACO2)
Aconitase 2 is the mitochondrial form of aconitase, an enzyme that catalyses the stereo-specific isomerization of citrate to isocitrate via cis-aconitate in the tricarboxylic acid cycle.

Adenine nucleotide translocase (ANT)
ANT is a reversible transporter of ATP and ADP. During OXPHOS the protein exchanges ATP out for ADP in, but when it becomes important to maintain a membrane potential, at times when OXPHOS is not working, ANT can work in reverse.

Alpha-ketoglutarate dehydrogenase (OGDH)
OGDH is located in the mitochondrial matrix, which catalyzes the decarboxylation of alpha ketoglutarate to produce succinyl CoA with reduction of NAD+ to generate NADH.

Apoptosis-inducing factor (AIF)
AIF is a flavoprotein localized in the inter-membrane space. It is a multi-functional protein with a vital oxidoreductase function, an anti-oxidant activity, an assembly function in Complex I formation, and an apoptogenic function.

Bile acid CoA amino acid N-acyltransferase (BAAT)
BAAT is a cytoplasmic monomeric enzyme found in liver tissue, gallbladder mucosa and pancreas which is involved in bile acid metabolism.

ATP synthase
ATP synthase is responsible for ATP production in oxidative phosphorylation and can work in reverse as a proton pumping ATPase.

Carbamoyl-phosphate synthase I (CPS1)
CPS is involved in the urea cycle where the enzyme plays an important role in removing excess ammonia from the cell.

Carnitine palmitoyltransferase 2 (CPT2)
Carnitine is attached to long chain fatty acids at the mitochondrial outer membrane by the enzyme CPT1.

Catalase
Catalse is a peroxisomal homotetrameric enzyme that has a detoxification role by catalyzing the decomposition of hydrogen peroxide to water and oxygen.

Complex I
Complex I catalyses electron entry from NADH via a flavin (FMN) and several non-heme iron centers.

Complex II
Complex II is both a component of the electron transport chain and an enzyme of the Krebs cycle.

Complex III
Complex III catalyses electron transfer from reduced ubiquinone or coenzyme Q 10 to cytochrome c.

Complex IV
Complex IV is the last enzyme in the respiratory electron transport chain and it helps to establish a transmembrane difference of proton electrochemical potential that the ATP synthase then uses to synthesize ATP.

Cyclophilin D
Cyclophilin D is the binding site for cyclosporins and this protein may be the gate for PTP.

Cytochrome c
Cytochrome c plays crucial roles in both oxidative phosphorylation and in apoptosis.

Delta(3,5)-delta(2,4)-dienoyl-CoA isomerase (ECH1)
ECH1 is a member of the hydratase/isomerase superfamily involved in fatty acid metabolism.

Delta-1-pyrroline-5-carboxylate dehydrogenase (ALDH4A1)
ALDH4A1 belongs to the aldehyde dehydrogenase family of proteins.

Dicarbonyl/L-xylulose reductase (DCXR)
DCXR is an oxidoreductase enzyme that catalyzes the NADH-dependent reduction of several pentoses, tetroses, alpha-dicarbonyl compounds and L-xylulose.

Epoxide hydrolase 1 (EPHX1)
Epoxide hydrolases (also known as epoxide hydratases) function in detoxification during drug metabolism.

Frataxin
The specific function of frataxin is still unknown, but it has been shown to play a role in iron metabolism.

Fumarase (FH)
The specific function of frataxin is still unknown, but it has been shown to play a role in iron metabolism.

Glyceraldehyde 3-phosphate dehydrogenase (GAPDH)
Glyceraldehyde 3-phosphate dehydrogenase (GAPDH) is an enzyme of the glycolytic pathway responsible for the conversion of glyceraldehyde 3- phosphate to D glycerate 1,3, -bisphosphate. It is present in high levels in all cells and is often used as a protein normalizing control.

Hydroxymethylglutaryl-CoA lyase (HMGCL)
HMGCL is a mitochondrial enzyme that is involved in the catabolism of branched amino acids such as leucine.

Hydroxysteroid dehydrogenase-like protein 2 (HSDL2)
HSDL2 belongs to the short-chain dehydrogenases/reductases (SDR) family. It is an as yet poorly described peroxisomal protein whose location suggests a role in fatty acid metabolism.

Medium-chain acyl CoA dehydrogenase (MCAD)
MCAD is an oxidoreductase enzyme of the mitochondrial fatty acid beta-oxidation pathway that is specific for acyl chain lengths of 4 to 16.

Mitofilin (MF)
Mitofilin has critical functions in mitochondrial morphology and mitochondrial fusion and fission, specifically in the formation of tubular cristae and cristae junctions.

Monoamine oxidase A (MAOA)
Catalyzes the oxidative deamination of biogenic and xenobiotic amines and has important functions in the metabolism of neuroactive and vasoactive amines in the central nervous system and peripheral tissues. MAOA preferentially oxidizes biogenic amines such as 5-hydroxytryptamine (5-HT), norepinephrine and epinephrine.

Monoamine oxidase B (MAOB)
Catalyzes the oxidative deamination of biogenic and xenobiotic amines and has important functions in the metabolism of neuroactive and vasoactive amines in the central nervous system and peripheral tissues. MAOB preferentially degrades benzylamine and phenylethylamine.

Nicotinamide nucleotide transhydrogenase (NNT)
NNT is a mitochondrial inner membrane protein catalyzing the interconversion of NADH and NADPH within the matrix space of the organelle.

Nitrotyrosine
Nitrotyrosine is formed on protein tyrosine residues by peroxynitrite-induced nitration and is considered a sensitive marker for oxidative stress.

Peroxisomal bifunctional enzyme (ECHD)
ECHD (gene name EHHADH), also called L-PBE, is one of two multifunctional proteins involved in peroxisomal fatty acid oxidation

Peroxisomal multifunctional enzyme 2 (MFE2)
MFE2 is involved in the catalysis of the D-stereoisomers of long chain fatty acids (C24 and above) as well as beta-oxidation of branched chain fatty acids such as pristanic acid.

Porin (VDAC)
The channel-forming protein porin (also called the voltage dependent anion channel, VDAC) is involved in both aerobic metabolism and apoptotic processes.

Pyruvate dehydrogenase (PDH)
The pyruvate dehydrogenase complex (PDH) is at the centre of aerobic carbohydrate metabolism.

Ribonuclease UK114 (HRSP12)
Endoribonuclease responsible for the inhibition of the translation by cleaving mRNA. Inhibits cell-free protein synthesis. Cleaves phosphodiester bonds only in single-stranded RNA.

Short-chain 3-hydroxyacyl-CoA dehydrogenase (SCHAD)
Plays an essential role in the mitochondrial beta-oxidation of short chain fatty acids. Exerts it highest activity toward 3-hydroxybutyryl-CoA.

Sirtuin 1 (SIRT1)
Plays an essential role in the mitochondrial beta-oxidation of short chain fatty acids. Exerts it highest activity toward 3-hydroxybutyryl-CoA.

SMAC/DIABLO
SMAC/DIABLO is an inhibitor of a class of proteins involved in cell death called the IAPs or inhibitor of apoptosis proteins.

Superoxide dismutase 2 (SOD2)
The principle cellular anti-oxidants are the superoxide dismutase family (SOD).

Trifunctional protein (TFP)
TFP is part of the mitochondrial fatty acid beta-oxidation pathway. It is located in the inner mitochondrial membrane and it catalyzes three out of the four steps in the beta-oxidation cycle with a specific affinity for long chain substrates.

Very-long-chain acyl CoA dehydrogenase (VLCAD)
A mitochondrial inner membrane enzyme, VLCAD is active toward esters of long-chain and very long chain fatty acids such as palmitoyl-CoA, mysritoyl-CoA and stearoyl-CoA.