MitoSciences: Proteins

All Proteins

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

60 kDa heat shock protein, mitochondrial (HSP60)
Facilitates the correct folding of imported proteins into the mitochondrion.

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

Acetyl-CoA acyltransferase, mitochondrial (ACAA2)
ACAA2 is involved in mitochondrial fatty acid oxidation.

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

Aconitase (ACO2)
Aconitase 2 is the mitochondrial form of aconitase and a key enzyme of the tricarboxylic acid (Krebs) cycle.

Adenine nucleotide translocase (ANT)
ANT is an exchanger (transporter) of ATP for ADP across the mitochondrial inner membrane.

Aldehyde dehydrogenase, mitochondrial (ALDH2)
ALDH2 belongs to the aldehyde dehydrogenase family of enzymes which catalyze the chemical transformation from acetaldehyde to acetic acid. It is the second enzyme of the major oxidative pathway of alcohol metabolism.

Alkaline phosphatase, Repressible
Phosphatase with broad substrate specificity. A truncated (soluble) version of the protein is responsible for the production of (E,E)-farnesol from (E,E)-farnesyl diphosphate.

Alpha-enolase (ENO1)
Multifunctional enzyme that, as well as its role in glycolysis, plays a part in various processes such as growth control, hypoxia tolerance and allergic responses.

Alpha-ketoglutarate dehydrogenase, OGDH (DLST)
OGDH is a mitochondrial matrix enzyme of the tricarboxylic acid (Krebs) cycle.

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.

Aspartate aminotransferase, mitochondrial (GOT2)
Plays a key role in amino acid metabolism. Important for metabolite exchange between mitochondria and cytosol. Facilitates cellular uptake of long-chain free fatty acids.

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

B-cell lymphoma/leukemia 11B (BCL11B/CTIP2)
BCL11B or CTIP2 is a tumor-suppressor protein involved in T-cell lymphomas.

B-cell receptor-associated protein 31 (BCAP31)
BCAP31 plays a role in anterograde transport of membrane proteins from the endoplasmic reticulum to the Golgi. May be involved in CASP8-mediated apoptosis.

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

Calnexin (CANX)
Calcium-binding protein that interacts with newly synthesized glycoproteins in the endoplasmic reticulum.

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.

Carboxylesterase 1 (CES1)
CES is involved in the detoxification of xenobiotics and in the activation of ester and amide prodrugs and so play a major role as determinants of pharmacokinetic behavior for most therapeutic agents containing an ester.

Carboxypeptidase Y
Involved in degradation of small peptides. Digests preferentially peptides containing an aliphatic or hydrophobic residue in P1' position, as well as methionine, leucine or phenylalanine in P1 position of ester substrate.

Carnitine palmitoyltransferase 2 (CPT2)
CPT2 releases long chain fatty acids that have been transported across the mitochondrial inner membrane attached to carnitine.

Catalase
Catalase is a peroxisomal enzyme that detoxifies potential reducing compounds by electron transfer to hydrogen peroxide to form water and oxygen.

Citrate Synthase (CS)
Citrate synthase (CS) is the initial enzyme of the tricarboxylic acid (TCA) cycle

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

Complex II (succinate dehydrogenase)
Complex II is both a component of the electron transport chain, and an enzyme of the tricarboxylic acid (Krebs) cycle. It catalyses electron entry via succinate into oxidative phosphorylation.

Complex III (ubiquinol cytochrome c oxidoreductase)
Complex III is the enzyme in oxidative phosphorylation that transfers electrons from reduced ubiquinone or coenzyme Q 10 to cytochrome c.

Complex IV (cytochrome c oxidase)
Complex IV is the last enzyme in the respiratory electron transport chain of oxidative phosphorylation. It catalyzes the transfer of electrons to molecular oxygen.

Creatine kinase M-type (CKM)
Creatine kinase, also known as creatine phosphokinase (CPK) or phospho-creatine kinase, is an enzyme expressed by various tissues and cell types.

Cyclophilin D
Cyclophilin D is the binding site for cyclosporins and this protein may be involved in the gating of the 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.

Diablo homolog, mitochondrial (SMAC)
SMAC/DIABLO is an inhibitor of a class of proteins involved in cell death called the IAPs or inhibitor of apoptosis 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.

Dolichol-phosphate mannosyltransferase
Transfers mannose from GDP-mannose to dolichol monophosphate to form dolichol phosphate mannose (Dol-P-Man) which is the mannosyl donor in pathways leading to N-glycosylation, glycosyl phosphatidylinositol membrane anchoring, and O-mannosylation of proteins.

Dynamin-like 120 kDa protein, mitochondrial (OPA1)
OPA1 is a dynamin-related GTPase required for mitochondrial fusion and regulation of apoptosis.

Electron transfer flavoprotein, mitochondrial (ETF)
The electron transfer flavoprotein serves as a specific electron acceptor for several dehydrogenases.

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

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

Fumarase (FH)
Fumarase is a mitochondrial enzyme involved in the tricarboxylic acid (Krebs) cycle.

Glucose-6-phosphate isomerase (G6PI)
Glucose-6-phosphate isomerase is a catalyzes the conversion of glucose-6-phosphate into fructose-6-phosphate in the second step of glycolysis.

Glutaryl-CoA dehydrogenase, mitochondrial (GCDH)
Glutaryl-CoA dehydrogenase is an acyl dehydrogenase involved in the metabolism of lysine, hydroxylysine, and tryptophan.

Glyceraldehyde 3-phosphate dehydrogenase (GAPDH)
GAPDH is an enzyme of the glycolytic pathway that is present in high levels in all cells and is often used as a protein normalizing control.

Glycogen synthase kinase-3 beta (GSK3B)
Constitutively active protein kinase that acts as a negative regulator in the hormonal control of glucose homeostasis, Wnt signaling and regulation of transcription factors and microtubules.

Green Fluorescent Protein (GFP)
Green fluorescence protein (GFP) is a 27 kDa protein derived from the jellyfish Aequorea victoria, which emits green light (emission peak at a wavelenth of 509 nm) when excited by blue light (excitation peak at a wavelenth of 395 nm).

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

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.

Hypoxia inducible factor 1 alpha (HIF1A)
The HIF1A gene encodes the HIF-1alpha subunit of hypoxia-inducible factor 1, a transcription factor that is essential for cardiovascular development and systemic O2 homeostasis. HIF-1 is found in mammalian cells cultured under reduced O2 tension and is necessary for transcriptional activation mediated by the erythropoietin gene enhancer in hypoxic cells.

L-lactate dehydrogenase B chain (LDHB)
Lactate dehydrogenase catalyzes the interconversion of pyruvate and lactate with concomitant interconversion of NADH and NAD+.

Malate dehydrogenase, mitochondrial (MDH2)
MDH2 is an enzyme in the citric acid cycle that catalyzes the conversion of malate into oxaloacetate (using NAD+) and vice versa.

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.

Microsomal glutathione S-transferase 3 (MGST3)
MGST3 catalyzes the conjugation of leukotriene A4 and reduced glutathione to produce leukotriene C4.

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.

Ornithine carbamoyltransferase, mitochondrial
OTC is involved in the urea cycle where the enzyme plays an important role in removing excess ammonia from the cell.

PDH kinases (PDK) and PDH phosphatases (PDP)
The pyruvate dehydrogenase complex (PDH) is regulated by phosphorylation at three Serine sites in the E1α subunit. Phosphoryaltion is controlled by four kinase isozymes (PDK1-4) and dephosphorylation by two phosphatase isozymes (PDP1,2) which are distributed differently in tissues.

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.

Peroxisome proliferator-activated receptor gamma coactivator 1-alpha (PGC1A)
Transcriptional coactivator for steroid receptors and nuclear receptors. Greatly increases the transcriptional activity of PPARG and thyroid hormone receptor on the uncoupling protein promoter. Can regulate key mitochondrial genes that contribute to the program of adaptive thermogenesis.

Phosphoglycerate kinase (PGK1)

Poly [ADP-ribose] polymerase 1 (PARP-1)
PARP-1 is nuclear DNA repair enzyme as component of base excision repair complex. In apoptosis, PARP-1 is cleaved by activated caspase-3. The proteolysis of PARP-1 through this cleavage renders the enzyme inactive and this further facilitates apoptotic cell death.

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 carboxylase, mitochondrial (PC)
PC catalyzes a 2-step reaction, involving the ATP-dependent carboxylation of the covalently attached biotin cofactor in the first step and the transfer of this carboxyl group to pyruvate in the second making oxaloacetate.

Pyruvate dehydrogenase (PDH)
The pyruvate dehydrogenase complex (PDH) is at the centre of aerobic carbohydrate metabolism through conversion of pyruvate to acetyl CoA for entry into the tricarboxylic acid (Krebs) cycle.

Quinone oxidoreductase (CRYZ)
CRYZ has been shown to have a novel NADPH:quinone oxidoreductase activity and is present in a variety of tissues from various mammals, particularly in the eye where it contributes to the lens protein mass.

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.

Serine-pyruvate aminotransferase (AGXT)
In peroxisomes, AGXT uses PLP (pyridoxal 5'-phosphate) as a cofactor and catalyses the nearly irreversible transfer of the amino group from alanine to glyoxylate, to yield glycine and pyruvate.

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.

Short-chain specific acyl-CoA dehydrogenase, mitochondrial (SCAD)
Acyl-CoA dehydrogenases are a class of enzymes that catalyze the initial step in each cycle of fatty acid β-oxidation in the mitochondria of cells.

Sirtuin 1 (SIRT1)
SIRT1 is a key deacetylase that is found in the cytosol and nucleus of the cell.

SRA stem-loop-interacting RNA-binding protein, mitochondrial (SLIRP)
SLIRP is a RNA binding protein that acts as a nuclear receptor corepressor.

Superoxide dismutase, mitochondrial (SOD2)
The principle cellular anti-oxidants are the superoxide dismutase family. SOD2 is the mitochondrial isoform of this enzyme.

Syntaxin PEP12
Plays a role in the sorting and targeting of vacuolar proteases.

Transcription factor A, mitochondrial (mtTFA)
mtTFA binds to the mitochondrial light strand promoter and functions in mitochondrial transcription regulation.

Transitional endoplasmic reticulum ATPase (VCP)
VCP is necessary for the fragmentation of Golgi stacks during mitosis and for their reassembly after mitosis.

Transketolase (TKT)
Transketolase is a key enzyme in the non-oxidative branch of the pentose phosphate pathway.

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.

Tropomyosin alpha-3 chain (TPM3)
Binds to actin filaments in muscle and non-muscle cells. Plays a central role, in association with the troponin complex, in the calcium dependent regulation of vertebrate striated muscle contraction. Smooth muscle contraction is regulated by interaction with caldesmon. In non-muscle cells is implicated in stabilizing cytoskeleton actin filaments.

V-type proton ATPase catalytic subunit A (VMA1)
Catalytic subunit of the peripheral V1 complex of vacuolar ATPase.

V-type proton ATPase subunit a, vacuolar isoform (VPH1)
Subunit of the integral membrane V0 complex of vacuolar ATPase essential for assembly and catalytic activity. Is present only in vacuolar V-ATPase complexes.

V-type proton ATPase subunit B (VMA2)
Non-catalytic subunit of the peripheral V1 complex of vacuolar ATPase.

Vacuolar protein sorting/targeting protein VPS10
Functions as a sorting receptor in the Golgi compartment required for the intracellular sorting and delivery of soluble vacuolar proteins, like carboxypeptidase Y (CPY) and proteinase A.

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.