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Catalogus nummer: (BOSSBS-9634R-CY7)
Leverancier: Bioss
Omschrijving: The Isocitrate dehydrogenase cytoplasmic enzyme is a homodimer of 416 residues that belongs to the isocitrate and isopropylmalate dehydrogenases family. IDHC catalyzes the third step of the citric acid cycle, which involves the oxidative decarboxylation of isocitrate, forming ?ketoglutarate and CO2 in a two step reaction. The first step involves the oxidation of isocitrate to the intermediate oxalosuccinate, while the second step involves the production of ?ketoglutarate. During this process, either NADH or NADPH is produced along with CO2. Ca2+ can bind to IDHC as a complex with isocitrate, acting as a competitive inhibitor of Mg2+. The IDHC enzyme is inactivated by phosphorylation at Ser-113 and contains a clasp-like domain wherein both polypeptide chains in the dimer interlock. IDHC is expressed in a wide range of species and also in organisms that lack a complete citric acid cycle.
UOM: 1 * 100 µl


Catalogus nummer: (BOSSBS-4192R-A350)
Leverancier: Bioss
Omschrijving: SLC25A20 is one of several closely related mitochondrial membrane carrier proteins that shuttle substrates between cytosol and the intramitochondrial matrix space. It mediates the transport of acylcarnitines into the mitochondrial matrix for their oxidation by the mitochondrial fatty acid oxidation pathway. Mutations in this gene are associated with carnitine acylcarnitine translocase deficiency, which can cause a variety of pathological conditions such as hypoglycemia, cardiac arrest, hepatomegaly, hepatic dysfunction and muscle weakness, and is usually lethal in new born and infants.
UOM: 1 * 100 µl


Catalogus nummer: (BOSSBS-8339R-A488)
Leverancier: Bioss
Omschrijving: The pantothenate kinase (PANK) family of proteins catalyzes the first step in coenzyme A (CoA) biosynthesis. Coenzyme A is an important coenzyme involved in the synthesis and oxidation of fatty acids, as well as the oxidation of pyruvate in the citric acid (Krebs) cycle. Pantothenate kinase 3 (PANK3) is a 370 amino acid member of the pantothenate kinase family that plays a role in the physiological regulation of the intracellular CoA concentration. Localized to the cytoplasm, PANK3 is regulated by feedback inhibition by CoA and its thioesters. PANK3 transfers a phosphate from ATP to pantothenate (Vitamin B5), resulting in formation of 4’-phosphopantothenate. Closely related to its family members, PANK1, PANK2 and PANK4, PANK3 is highly expressed in liver. Pantothenate kinase associated neurodegeneration (PKAN) results from mutations in the gene encoding PANK2, the only mitochondria targeted human PANK.
UOM: 1 * 100 µl


Catalogus nummer: (BOSSBS-9634R-CY5)
Leverancier: Bioss
Omschrijving: The Isocitrate dehydrogenase cytoplasmic enzyme is a homodimer of 416 residues that belongs to the isocitrate and isopropylmalate dehydrogenases family. IDHC catalyzes the third step of the citric acid cycle, which involves the oxidative decarboxylation of isocitrate, forming ?ketoglutarate and CO2 in a two step reaction. The first step involves the oxidation of isocitrate to the intermediate oxalosuccinate, while the second step involves the production of ?ketoglutarate. During this process, either NADH or NADPH is produced along with CO2. Ca2+ can bind to IDHC as a complex with isocitrate, acting as a competitive inhibitor of Mg2+. The IDHC enzyme is inactivated by phosphorylation at Ser-113 and contains a clasp-like domain wherein both polypeptide chains in the dimer interlock. IDHC is expressed in a wide range of species and also in organisms that lack a complete citric acid cycle.
UOM: 1 * 100 µl


Catalogus nummer: (BOSSBS-5015R-A647)
Leverancier: Bioss
Omschrijving: The protein encoded by this gene belongs to the acyl-CoA dehydrogenase family, which is a family of mitochondrial flavoenzymes involved in fatty acid and branched chain amino-acid metabolism. This protein is one of the four enzymes that catalyze the initial step of mitochondrial beta-oxidation of straight-chain fatty acid. Defects in this gene are the cause of long-chain acyl-CoA dehydrogenase (LCAD) deficiency, leading to nonketotic hypoglycemia. [provided by RefSeq].
UOM: 1 * 100 µl


Catalogus nummer: (BOSSBS-8339R)
Leverancier: Bioss
Omschrijving: The pantothenate kinase (PANK) family of proteins catalyzes the first step in coenzyme A (CoA) biosynthesis. Coenzyme A is an important coenzyme involved in the synthesis and oxidation of fatty acids, as well as the oxidation of pyruvate in the citric acid (Krebs) cycle. Pantothenate kinase 3 (PANK3) is a 370 amino acid member of the pantothenate kinase family that plays a role in the physiological regulation of the intracellular CoA concentration. Localized to the cytoplasm, PANK3 is regulated by feedback inhibition by CoA and its thioesters. PANK3 transfers a phosphate from ATP to pantothenate (Vitamin B5), resulting in formation of 4’-phosphopantothenate. Closely related to its family members, PANK1, PANK2 and PANK4, PANK3 is highly expressed in liver. Pantothenate kinase associated neurodegeneration (PKAN) results from mutations in the gene encoding PANK2, the only mitochondria targeted human PANK.
UOM: 1 * 100 µl


Catalogus nummer: (BOSSBS-4047R-CY7)
Leverancier: Bioss
Omschrijving: ACADM protein is a medium chain specific (C4 to C12 straight chain) acyl Coenzyme A dehydrogenase. The enzyme catalyzes the initial step of the mitochondrial fatty acid beta oxidation pathway. ACADM expression is induced during periods of fasting, when reliance on fatty acids for energy is increased. Clinical phenotypes are associated with ACADM hereditary deficiency.
UOM: 1 * 100 µl


Catalogus nummer: (BOSSBS-4047R-CY3)
Leverancier: Bioss
Omschrijving: ACADM protein is a medium chain specific (C4 to C12 straight chain) acyl Coenzyme A dehydrogenase. The enzyme catalyzes the initial step of the mitochondrial fatty acid beta oxidation pathway. ACADM expression is induced during periods of fasting, when reliance on fatty acids for energy is increased. Clinical phenotypes are associated with ACADM hereditary deficiency.
UOM: 1 * 100 µl


Catalogus nummer: (BOSSBS-11797R-A350)
Leverancier: Bioss
Omschrijving: Aldehyde dehydrogenases (ALDHs) mediate the NADP+-dependent oxidation of aldehydes into acids and play an important role in the detoxification of alcohol-derived acetaldehyde, as well as in lipid peroxidation and in the metabolism of corticosteroids, biogenic amines and neurotransmitters. ALDH3A2 (aldehyde dehydrogenase 3 family, member A2), also known as SLS, FALDH or ALDH10, is a 485 amino acid single-pass membrane protein that localizes to the cytoplasmic side of the endoplasmic reticulum and belongs to the aldehyde dehydrogenase family. Expressed in a variety of tissues, including liver, heart, lung, brain, kidney and placenta, ALDH3A2 catalyzes the NAD+-dependent oxidation of long-chain aliphatic aldehydes to fatty acids, a process that is necessary for detoxification and lipid metabolism. Defects in the gene encoding ALDH3A2 are the cause of Sjoegren-Larsson syndrome (SLS), an autosomal recessive neurocutaneous disorder characterized by severe mental retardation, seizures and speech defects. Multiple isoforms of ALDH3A2 exist due to alternative splicing events.
UOM: 1 * 100 µl


Catalogus nummer: (BOSSBS-4047R-A488)
Leverancier: Bioss
Omschrijving: ACADM protein is a medium chain specific (C4 to C12 straight chain) acyl Coenzyme A dehydrogenase. The enzyme catalyzes the initial step of the mitochondrial fatty acid beta oxidation pathway. ACADM expression is induced during periods of fasting, when reliance on fatty acids for energy is increased. Clinical phenotypes are associated with ACADM hereditary deficiency.
UOM: 1 * 100 µl


Catalogus nummer: (BOSSBS-0250R-A555)
Leverancier: Bioss
Omschrijving: Ligand-activated transcription factor. Receptor that binds peroxisome proliferators such as hypolipidemic drugs and fatty acids. Has a preference for poly-unsaturated fatty acids, such as gamma-linoleic acid and eicosapentanoic acid. Once activated by a ligand, the receptor binds to promoter elements of target genes. Regulates the peroxisomal beta-oxidation pathway of fatty acids. Functions as transcription activator for the acyl-CoA oxidase gene. Decreases expression of NPC1L1 once activated by a ligand (By similarity).
UOM: 1 * 100 µl


Catalogus nummer: (BOSSBS-0250R-A680)
Leverancier: Bioss
Omschrijving: Ligand-activated transcription factor. Receptor that binds peroxisome proliferators such as hypolipidemic drugs and fatty acids. Has a preference for poly-unsaturated fatty acids, such as gamma-linoleic acid and eicosapentanoic acid. Once activated by a ligand, the receptor binds to promoter elements of target genes. Regulates the peroxisomal beta-oxidation pathway of fatty acids. Functions as transcription activator for the acyl-CoA oxidase gene. Decreases expression of NPC1L1 once activated by a ligand (By similarity).
UOM: 1 * 100 µl


Catalogus nummer: (BOSSBS-0250R-A350)
Leverancier: Bioss
Omschrijving: Ligand-activated transcription factor. Receptor that binds peroxisome proliferators such as hypolipidemic drugs and fatty acids. Has a preference for poly-unsaturated fatty acids, such as gamma-linoleic acid and eicosapentanoic acid. Once activated by a ligand, the receptor binds to promoter elements of target genes. Regulates the peroxisomal beta-oxidation pathway of fatty acids. Functions as transcription activator for the acyl-CoA oxidase gene. Decreases expression of NPC1L1 once activated by a ligand (By similarity).
UOM: 1 * 100 µl


Catalogus nummer: (BOSSBS-4192R-A680)
Leverancier: Bioss
Omschrijving: SLC25A20 is one of several closely related mitochondrial membrane carrier proteins that shuttle substrates between cytosol and the intramitochondrial matrix space. It mediates the transport of acylcarnitines into the mitochondrial matrix for their oxidation by the mitochondrial fatty acid oxidation pathway. Mutations in this gene are associated with carnitine acylcarnitine translocase deficiency, which can cause a variety of pathological conditions such as hypoglycemia, cardiac arrest, hepatomegaly, hepatic dysfunction and muscle weakness, and is usually lethal in new born and infants.
UOM: 1 * 100 µl


Catalogus nummer: (ENZOALX380011M050)
Leverancier: ENZO LIFE SCIENCES
Omschrijving: Antibiotic which suppresses nitric oxide (NO) toxicity in pancreatic islet cells. Inhibits protein synthesis by inhibition of elongation factor G at the level of the ribosome.
UOM: 1 * 50 mg


Catalogus nummer: (BOSSBS-4192R-A647)
Leverancier: Bioss
Omschrijving: SLC25A20 is one of several closely related mitochondrial membrane carrier proteins that shuttle substrates between cytosol and the intramitochondrial matrix space. It mediates the transport of acylcarnitines into the mitochondrial matrix for their oxidation by the mitochondrial fatty acid oxidation pathway. Mutations in this gene are associated with carnitine acylcarnitine translocase deficiency, which can cause a variety of pathological conditions such as hypoglycemia, cardiac arrest, hepatomegaly, hepatic dysfunction and muscle weakness, and is usually lethal in new born and infants.
UOM: 1 * 100 µl


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