Descrizione: LMBRD1 Antibody: LMBRD1, also known as NESI (nuclear export signal-interacting protein, is a lysosomal membrane protein that is thought be involved in the transport and metabolism of cobalamin. LMBRD1 was initially identified as interacting with the large form of the hepatitis delta antigen and may be required for the nucleocytoplasmic shuttling of the hepatitis delta virus. Mutations in this gene are associated with the vitamin B12 metabolism disorder termed, homocystinuria-megaloblastic anemia complementation type F (cblF).

Codice catalogo: PRSI6047

UOM: 1 * 100 µG

Codice articolo fornitore: 6047

Codice articolo locale: PRSI6047

Fornitore: ProSci Inc.

Promo

Descrizione: ACADM Is the medium-chain specific (C4 to C12 straight chain) acyl-Coenzyme A dehydrogenase. The homotetramer enzyme catalyzes the initial step of the mitochondrial fatty acid beta-oxidation pathway. Clinical phenotypes are associated with ACADM hereditary deficiency.This gene encodes the medium-chain specific (C4 to C12 straight chain) acyl-Coenzyme A dehydrogenase. The homotetramer enzyme catalyzes the initial step of the mitochondrial fatty acid beta-oxidation pathway. Defects in this gene cause medium-chain acyl-CoA dehydrogenase deficiency, a disease characterized by hepatic dysfunction, fasting hypoglycemia, and encephalopathy, which can result in infantile death. Alternatively spliced transcript variants encoding different isoforms have been found for this gene.

Codice catalogo: PRSI25-092

UOM: 1 * 50 µG

Codice articolo fornitore: 25-092

Codice articolo locale: PRSI25-092

Fornitore: ProSci Inc.

Promo

Descrizione: Acetyl-Coenzyme A acetyltransferase 2 is an enzyme involved in lipid metabolism. Reported patients with ACAT2 deficiency have shown severe mental retardation and hypotonus. The ACAT2 gene shows complementary overlapping with the 3-prime region of the TCP1 gene in both mouse and human. These genes are encoded on opposite strands of DNA, as well as in opposite transcriptional orientation.

Codice catalogo: PRSI27-710

UOM: 1 * 100 µG

Codice articolo fornitore: 27-710

Codice articolo locale: PRSI27-710

Fornitore: ProSci Inc.

Promo

Descrizione: Acetyl-Coenzyme A acetyltransferase 2 is an enzyme involved in lipid metabolism. Reported patients with ACAT2 deficiency have shown severe mental retardation and hypotonus. The ACAT2 gene shows complementary overlapping with the 3-prime region of the TCP1 gene in both mouse and human. These genes are encoded on opposite strands of DNA, as well as in opposite transcriptional orientation.

Codice catalogo: PRSI27-709

UOM: 1 * 100 µG

Codice articolo fornitore: 27-709

Codice articolo locale: PRSI27-709

Fornitore: ProSci Inc.

Promo

Descrizione: N-alpha-acetylation is one of the most common protein modifications that occurs during protein synthesis and involves the transfer of an acetyl group from acetyl-coenzyme A to the protein alpha-amino group. ARD1A, together with NATH (NARG1; MIM 608000), is part of a major N-alpha-acetyltransferase complex responsible for alpha-acetylation of proteins and peptides.N-alpha-acetylation is one of the most common protein modifications that occurs during protein synthesis and involves the transfer of an acetyl group from acetyl-coenzyme A to the protein alpha-amino group. ARD1A, together with NATH (NARG1; MIM 608000), is part of a major N-alpha-acetyltransferase complex responsible for alpha-acetylation of proteins and peptides (Sanchez-Puig and Fersht, 2006 [PubMed 16823041]).

Codice catalogo: PRSI26-327

UOM: 1 * 50 µG

Codice articolo fornitore: 26-327

Codice articolo locale: PRSI26-327

Fornitore: ProSci Inc.

Promo

Descrizione: ACADVL is targeted to the inner mitochondrial membrane where it catalyzes the first step of the mitochondrial fatty acid beta-oxidation pathway. This acyl-Coenzyme A dehydrogenase is specific to long-chain and very-long-chain fatty acids. A deficiency in ACADVL protein reduces myocardial fatty acid beta-oxidation and is associated with cardiomyopathy.The protein encoded by this gene is targeted to the inner mitochondrial membrane where it catalyzes the first step of the mitochondrial fatty acid beta-oxidation pathway. This acyl-Coenzyme A dehydrogenase is specific to long-chain and very-long-chain fatty acids. A deficiency in this gene product reduces myocardial fatty acid beta-oxidation and is associated with cardiomyopathy. Alternative splicing results in multiple transcript variants encoding different isoforms.

Codice catalogo: PRSI26-880

UOM: 1 * 50 µG

Codice articolo fornitore: 26-880

Codice articolo locale: PRSI26-880

Fornitore: ProSci Inc.

Promo

Descrizione: A number of tumor cells and cell lines have been observed to have highly upregulated expression and activity of fatty acid synthase (FAS). Inhibition of FAS by the irreversible inhibitor cerulenin leads to cytotoxicity and apoptosis in human cancer cell lines, an effect believed to be mediated by the accumulation of malonyl-coenzyme A in cells with an upregulated FAS pathway. Cerulenin also leads to profound weight loss and feeding inhibition in both high-fat diet wild type obese and leptin-deficient ob/ob mice.

Codice catalogo: CAYM10005647-5

UOM: 1 * 5 mg

Codice articolo fornitore: 10005647-5

Codice articolo locale: CAYM10005647-5

Fornitore: Cayman Chemical

Promo

Descrizione: The coenzyme NAD and its derivatives are involved in hundreds of metabolic redox reactions and are utilized in protein ADP-ribosylation, histone deacetylation, and in some Ca (2+) signaling pathways. NMNAT (EC 2.7.7.1) is a central enzyme in NAD biosynthesis, catalyzing the condensation of nicotinamide mononucleotide (NMN) or nicotinic acid mononucleotide (NaMN) with the AMP moiety of ATP to form NAD or NaAD.The coenzyme NAD and its derivatives are involved in hundreds of metabolic redox reactions and are utilized in protein ADP-ribosylation, histone deacetylation, and in some Ca (2+) signaling pathways. NMNAT (EC 2.7.7.1) is a central enzyme in NAD biosynthesis, catalyzing the condensation of nicotinamide mononucleotide (NMN) or nicotinic acid mononucleotide (NaMN) with the AMP moiety of ATP to form NAD or NaAD (Zhang et al., 2003 [PubMed 12574164]).

Codice catalogo: PRSI30-467

UOM: 1 * 100 µG

Codice articolo fornitore: 30-467

Codice articolo locale: PRSI30-467

Fornitore: ProSci Inc.

Promo

Descrizione: D-Pantothenic acid is an essential vitamin (except in horses, ruminants). Pantothenic acid is involved in a number of biological reactions including: A precursor in the biosynthesis of coenzyme A, production of energy, catabolism of fatty acids and amino acids, synthesis of fatty acids, phospholipids, sphingolipids, cholesterol and steroid hormones, synthesis of heme and the neurotransmitter acetylcholine and involved in regulation of gene expression and in signal transduction.

Codice catalogo: ICNA0210122805

UOM: 1 * 5 g

Codice articolo fornitore: 0210122805

Codice articolo locale: ICNA0210122805

Fornitore: MP Biomedicals

Promo

Descrizione: The CD320 antigen gene encodes the transcobalamin receptor (TCBLR). Cellular uptake of cobalamin (vitamin B12) is mediated by receptors expressed on the cell surface. Transcobalamin II (TCN2; MIM 613441), a plasma protein secreted by endothelial cells, binds the cobalamin absorbed in the distal ileum and carries 10 to 30% of total circulating cobalamin. CD320 encodes a transcobalamin receptor that binds TCN2-cobalamin at the plasma membrane and internalizes the complex by endocytosis (Quadros et al., 2009 [PubMed 18779389]).

Codice catalogo: PRSI56-874

UOM: 1 * 400 µl

Codice articolo fornitore: 56-874

Codice articolo locale: PRSI56-874

Fornitore: ProSci Inc.

Promo

Descrizione: ACSL1 encodes an isozyme of the long-chain fatty-acid-coenzyme A ligase family. Although differing in substrate specificity, subcellular localization, and tissue distribution, all isozymes of this family convert free long-chain fatty acids into fatty acyl-CoA esters, and thereby play a key role in lipid biosynthesis and fatty acid degradation.The protein encoded by this gene is an isozyme of the long-chain fatty-acid-coenzyme A ligase family. Although differing in substrate specificity, subcellular localization, and tissue distribution, all isozymes of this family convert free long-chain fatty acids into fatty acyl-CoA esters, and thereby play a key role in lipid biosynthesis and fatty acid degradation. Publication Note: This RefSeq record includes a subset of the publications that are available for this gene. Please see the Entrez Gene record to access additional publications.

Codice catalogo: PRSI27-705

UOM: 1 * 50 µG

Codice articolo fornitore: 27-705

Codice articolo locale: PRSI27-705

Fornitore: ProSci Inc.

Promo

Descrizione: ECHS1 functions in the second step of the mitochondrial fatty acid beta-oxidation pathway. It catalyzes the hydration of 2-trans-enoyl-coenzyme A (CoA) intermediates to L-3-hydroxyacyl-CoAs. The protein is a member of the hydratase/isomerase superfamily. It localizes to the mitochondrial matrix. Transcript variants utilizing alternative transcription initiation sites have been described in the literature.The protein encoded by this gene functions in the second step of the mitochondrial fatty acid beta-oxidation pathway. It catalyzes the hydration of 2-trans-enoyl-coenzyme A (CoA) intermediates to L-3-hydroxyacyl-CoAs. The gene product is a member of the hydratase/isomerase superfamily. It localizes to the mitochondrial matrix. Transcript variants utilizing alternative transcription initiation sites have been described in the literature. Publication Note: This RefSeq record includes a subset of the publications that are available for this gene. Please see the Entrez Gene record to access additional publications.

Codice catalogo: PRSI26-068

UOM: 1 * 50 µG

Codice articolo fornitore: 26-068

Codice articolo locale: PRSI26-068

Fornitore: ProSci Inc.

Promo

Descrizione: ACAA1 is an enzyme operative in the beta-oxidation system of the peroxisomes. Deficiency of this enzyme leads to pseudo-Zellweger syndrome.Acetyl-Coenzyme A acyltransferase (ACAA1) is an enzyme operative in the beta-oxidation system of the peroxisomes. Deficiency of this enzyme leads to pseudo-Zellweger syndrome. Publication Note: This RefSeq record includes a subset of the publications that are available for this gene. Please see the Entrez Gene record to access additional publications.

Codice catalogo: PRSI26-292

UOM: 1 * 50 µG

Codice articolo fornitore: 26-292

Codice articolo locale: PRSI26-292

Fornitore: ProSci Inc.

Promo

Descrizione: ACSL1 encodes an isozyme of the long-chain fatty-acid-coenzyme A ligase family. Although differing in substrate specificity, subcellular localization, and tissue distribution, all isozymes of this family convert free long-chain fatty acids into fatty acyl-CoA esters, and thereby play a key role in lipid biosynthesis and fatty acid degradation.The protein encoded by this gene is an isozyme of the long-chain fatty-acid-coenzyme A ligase family. Although differing in substrate specificity, subcellular localization, and tissue distribution, all isozymes of this family convert free long-chain fatty acids into fatty acyl-CoA esters, and thereby play a key role in lipid biosynthesis and fatty acid degradation. Publication Note: This RefSeq record includes a subset of the publications that are available for this gene. Please see the Entrez Gene record to access additional publications.

Codice catalogo: PRSI27-706

UOM: 1 * 50 µG

Codice articolo fornitore: 27-706

Codice articolo locale: PRSI27-706

Fornitore: ProSci Inc.

Promo

Descrizione: L-Lactate Dehydrogenase A Chain (LDHA) is an enzyme that catalyzes the conversion of L-lactate and NAD+ to pyruvate and NADH in the final step of anaerobic glycolysis. LDHA contains an N-terminal coenzyme binding region, a central catalytic site, and at least nine utilized Lys acetylation and two Tyr phosphorylation sites. LDHA belongs to the lactate dehydrogenase family, expressed predominantly in muscle tissue. LDHA mutations have been linked to exertional myoglobinuria.

Codice catalogo: PRSI91-215

UOM: 1 * 50 µG

Codice articolo fornitore: 91-215

Codice articolo locale: PRSI91-215

Fornitore: ProSci Inc.

Promo

Descrizione: Uroporphyrinogen-III Synthase is an enzyme which belongs to the uroporphyrinogen-III synthase family. Uroporphyrinogen-III Synthase is ubiquitous and it is involved in Porphyrin metabolism. Porphyrins act as cofactors for a multitude of enzymes that perform a variety of processes within the cell such as Methionine synthesis (Vitamin B12) or oxygen transport (Heme). Uroporphyrinogen-III Synthase can catalyze cyclization of the linear Tetrapyrrole, Hydroxymethylbilane, to the Macrocyclic Uroporphyrinogen III, the branch point for the various sub-pathways leading to the wide diversity of Porphyrins. Defects in Uroporphyrinogen-III Synthase are the cause of Congenital Erythropoietic Porphyria (CEP).

Codice catalogo: PRSI91-248

UOM: 1 * 50 µG

Codice articolo fornitore: 91-248

Codice articolo locale: PRSI91-248

Fornitore: ProSci Inc.

Promo