MitoSciences: Proteins

All Proteins

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

5'-AMP-activated protein kinase catalytic subunit alpha-1
Catalytic subunit of AMP-activated protein kinase (AMPK), an energy sensor protein kinase that plays a key role in regulating cellular energy metabolism.

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.

Akt1 - RAC-alpha serine/threonine-protein kinase (AKT1)
AKT1 is one of 3 closely related serine/threonine-protein kinases (AKT1, AKT2 and AKT3) called the AKT kinase, and which regulate many processes including metabolism, proliferation, cell survival, growth and angiogenesis.

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.

Aldo-keto reductase family 1 member C1 (AKR1C1)
Converts progesterone to its inactive form, 20-alpha-dihydroxyprogesterone (20-alpha-OHP).

Aldolase B, Fructose-bisphosphate (ALDOB)
Aldolase B also known as fructose-bisphosphate aldolase B or liver-type aldolase is one of three isoenzymes (A, B, and C) of the class I fructose 1,6-bisphosphate aldolase enzyme, and plays a key role in both glycolysis and gluconeogenesis.

Alkaline phosphatase, Repressible (AP)
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 111 (BCL11A/CTIP1)
Functions as a myeloid and B-cell proto-oncogene. May play important roles in leukemogenesis and hematopoiesis.

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.

Bcl2 antagonist of cell death (BAD)
Bad promotes cell death in 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 (CPY)
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 O-palmitoyltransferase 1 (CPT1)
Carnitine palmitoyltransferase I is the first component and rate-limiting step of the carnitine palmitoyltransferase system in beta-oxidation of fatty acids.

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

Caspase 3
Caspase 3 is a cysteine protease involved in the activation cascade of caspases responsible for apoptosis execution.

Caspase-9 (CASP9)
Caspase 9 is involved in the activation cascade of caspases responsible for apoptosis execution.

Catalase (CAT)
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 (PPIF)
Cyclophilin D is the binding site for cyclosporins and this protein may be involved in the gating of the PTP.

Cytochrome c (CYC)
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.

Dolichol-phosphate mannosyltransferase (DPM1)
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.

Electron transfer flavoprotein-ubiquinone oxidoreductase, mitochondrial (ETFDH)
Accepts electrons from ETF and reduces ubiquinone in the mitochondrial inner membrane.

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.

GAL4 Protein
Protein is a positive regulator for the gene expression of the galactose-induced genes.

Glial fibrillary acidic protein
GFAP, a class-III intermediate filament, is a cell-specific marker that, during the development of the central nervous system, distinguishes astrocytes from other glial cells.

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.

Glutathione S-Transferase (GST)
GST (Glutathione S-Transferase) is a 26kDa protein encoded by the parasitic helminth Schistosoma japonicum and widely used in the pGEX family of GST plasmid expression vectors as a fusion protein with foreign proteins.

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).

Histone H2A.x (H2AFX)
Variant histone H2A which replaces conventional H2A in a subset of nucleosomes.

Histone H3
Histone H3 is one of the four core histone proteins (H2A, H2B, H3 and H4) that pack DNA in nucleosomes.

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.

Immunoglobulin G
There are five classes of mammalian immunoglobulins: IgA, IgD, IgE, IgM, and IgG. IgG is the most abundant immunoglobulin and is equally distributed in blood and tissue.

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

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

Long-chain specific acyl-CoA dehydrogenase, mitochondrial (ACADL)
Catalyze the initial step of mitochondrial beta-oxidation of straight-chain fatty acid.

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

Mitofusin-1 (MFN1)
Essential transmembrane GTPase, which mediates mitochondrial fusion.

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 (3NT)
Nitrotyrosine is formed on protein tyrosine residues by peroxynitrite-induced nitration and is considered a sensitive marker for oxidative stress.

Nucleolin (NCL)
Nucleolin is the major nucleolar protein found associated with intranucleolar chromatin and pre-ribosomal particles.

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

p53, Cellular tumor antigen (TP53)
p53 (TP53 gene) acts as a tumor suppressor in many tumor types and induces growth arrest or apoptosis depending on the physiological circumstances and cell type.

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.

Ribosomal protein S6 kinase alpha-1
RSK1p90 is a 90kDa ribosomal S6 kinase that belongs to a family of serine and threonine kinases. These kinases include a binding site for extracellular signal-related kinases (ERKs) at its carboxy-terminus.

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.

Serine/threonine-protein kinase mTOR
Serine/threonine protein kinase which is a central regulator of cellular metabolism, growth and survival in response to hormones, growth factors, nutrients, energy and stress signals.

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.

SMAC 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.

Sodium/potassium-transporting ATPase subunit alpha-1
Catalytic alpha subunit of the enzyme which catalyzes the hydrolysis of ATP coupled with the exchange of sodium and potassium ions across the plasma membrane.

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

Superoxide Dismutase 1 [Cu-Zn]
SOD1 or Superoxide dismutase [Cu-Zn] is an enzyme that binds copper and zinc ions and is one of three superoxide dismutases responsible for destroying free superoxide radicals in the body.

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

Synaptosomal-associated protein 25 (SNAP25)
The SNAP-25 protein is a component of the SNARE complex.

Synaptotagmin-2
Synaptotagmin-2 functions as a Ca2+ sensor for fast neurotransmitter release in caudal brain regions.

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.

UDP-glucuronosyltransferase (UGT)
UDPGT is of major importance in the conjugation and subsequent elimination of potentially toxic xenobiotics and endogenous compounds.

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.

Vimentin
Vimentins are class-III intermediate filaments found in various non-epithelial cells, especially mesenchymal cells. Vimentin is attached to the nucleus, endoplasmic reticulum, and mitochondria, either laterally or terminally