Human BCKDHA Protein

<strong>Human BCKDHA Protein</strong>_x000D_ <strong>Catalog number:</strong> B2011630_x000D_ <strong>Lot number:</strong> Batch Dependent_x000D_ <strong>Expiration Date:</strong> Batch dependent_x000D_ <strong>Amount:</strong> 10 µg_x000D_ <strong>Molecular Weight or Concentration:</strong> 0.25 mg/mL_x000D_ <strong>Supplied as:</strong> Solution_x000D_ <strong>Applications:</strong> molecular tool for various biochemical applications_x000D_ <strong>Storage:</strong> -20 °C_x000D_ <strong>Keywords:</strong> OVD1A protein, MSUD1 protein, BCKDH E1-alpha protein, BCKDE1A protein, 2-oxoisovalerate dehydrogenase subunit alpha protein, MSU protein_x000D_ <strong>Grade:</strong> Biotechnology grade. All products are highly pure. All solutions are made with Type I ultrapure water (resistivity &gt;18 MΩ-cm) and are filtered through 0.22 um._x000D_ _x000D_ <strong>References:</strong>_x000D_ 1: Lynch CJ, Adams SH. Branched-chain amino acids in metabolic signalling and insulin resistance Nat Rev Endocrinol. 2014 Dec;10(12):723-36._x000D_ 2: Kim JH, Kim J, Im SS, Lee JH, Hwang S, Chang EJ, Shin DM, Rho JK, Son J. BIX01294 inhibits EGFR signaling in EGFR-mutant lung adenocarcinoma cells through a BCKDHA-mediated reduction in the EGFR level Exp Mol Med. 2021 Dec;53(12):1877-1887._x000D_ 3: Campanholi DRR, Margutti AVB, Silva WA Jr, Garcia DF, Molfetta GA, Marques AA, Schwartz IVD, Cornejo V, Hamilton V, Castro G, Sperb-Ludwig F, Borges ES, Camelo JS Jr. Molecular basis of various forms of maple syrup urine disease in Chilean patients Mol Genet Genomic Med. 2021 May;9(5):e1616._x000D_ 4: Liu GY, Gao SZ. Molecular cloning, sequence identification and tissue expression profile of three novel sheep (Ovis aries) genes - BCKDHA, NAGA and HEXA Biol Res. 2009;42(1):69-77. Epub 2009 Jun 11._x000D_ 5: Chuang JL, Cox RP, Chuang DT. Characterization of the promoter-regulatory region and structural organization of E1 alpha gene (BCKDHA) of human branched-chain alpha-keto acid dehydrogenase complex J Biol Chem. 1993 Apr 15;268(11):8309-16._x000D_ 6: Medina MF, Castro G, Falcon F, Cabello JF, Faundes V, Ruffato D, Salazar MF, Arias C, Peñaloza F, De La Parra A, Cornejo V. Maple syrup urine disease: Characteristics of diagnosis and treatment in 45 patients in Chile Am J Med Genet C Semin Med Genet. 2021 Sep;187(3):373-380._x000D_ 7: Zeynalzadeh M, Tafazoli A, Aarabi A, Moghaddassian M, Ashrafzadeh F, Houshmand M, Taghehchian N, Abbaszadegan MR. Four novel mutations of the BCKDHA, BCKDHB and DBT genes in Iranian patients with maple syrup urine disease J Pediatr Endocrinol Metab. 2018 Jan 26;31(2):205-212._x000D_ 8: Lee JH, Cho YR, Kim JH, Kim J, Nam HY, Kim SW, Son J. Branched-chain amino acids sustain pancreatic cancer growth by regulating lipid metabolism Exp Mol Med. 2019 Nov 29;51(11):1-11._x000D_ 9: Pontoizeau C, Simon-Sola M, Gaborit C, Nguyen V, Rotaru I, Tual N, Colella P, Girard M, Biferi MG, Arnoux JB, Rötig A, Ottolenghi C, de Lonlay P, Mingozzi F, Cavazzana M, Schiff M. Neonatal gene therapy achieves sustained disease rescue of maple syrup urine disease in mice Nat Commun. 2022 Jun 7;13(1):3278._x000D_ <a href="https://pubmed.ncbi.nlm.nih.gov/19456321">10: Quental S, Gusmão A, Rodríguez-Pombo P, Ugarte M, Vilarinho L, Amorim A, Prata MJ. Revisiting MSUD in Portuguese Gypsies: evidence for a founder mutation and for a mutational hotspot within the BCKDHA gene Ann Hum Genet. 2009 May;73(Pt 3):298-303. </a>_x000D_ _x000D_ <strong>Products Related to Human BCKDHA Protein can be found at</strong> <a href="https://moleculardepot.com/product-category/Proteins/"> Proteins</a>