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Proteintech
BCKDHB Polyclonal Antibody
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Product Details
13685-1-AP
Species Reactivity
Published species
Host/Isotype
Class
Type
Immunogen
Conjugate
Form
Concentration
Purification
Storage buffer
Contains
Storage conditions
Shipping conditions
Product Specific Information
Immunogen sequence: TVEDAAQRR QVAHFTFQPD PEPREYGQTQ KMNLFQSVTS ALDNSLAKDP TAVIFGEDVA FGGVFRCTVG LRDKYGKDRV FNTPLCEQGI VGFGIGIAVT GATAIAEIQF ADYIFPAFDQ IVNEAAKYRY RSGDLFNCGS LTIRSPWGCV GHGALYHSQS PEAFFAHCPG IKVVIPRSPF QAKGLLLSCI EDKNPCIFFE PKILYRAAAE EVPIEPYNIP LSQAEVIQEG SDVTLVAWGT QVHVIREVAS MAKEKLGVSC EVIDLRTIIP WDVDTICKSV IKTGRLLISH EAPLTGGFAS EISSTVQEEC FLNLEAPISR VCGYDTPFPH IFEPFYIPDK WKCYDALRKM INY (41-392 aa encoded by BC040139)
Target Information
Branched-chain keto acid dehydrogenase is a multienzyme complex associated with the inner membrane of mitochondria, and functions in the catabolism of branched-chain amino acids. The complex consists of multiple copies of 3 components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3). This gene encodes the E1 beta subunit, and mutations therein have been associated with maple syrup urine disease (MSUD), type 1B, a disease characterized by a maple syrup odor to the urine in addition to mental and physical retardation, and feeding problems.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
Bioinformatics
Protein Aliases: 2-oxoisovalerate dehydrogenase beta subunit; 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial; BCKD BCKDH E1-beta; BCKDE1B; BCKDH E1-beta; branched chain alpha-ketoacid dehydrogenase E1-beta subunit; branched chain keto acid dehydrogenase E1 beta; branched chain keto acid dehydrogenase E1, beta polypeptide; Branched-chain alpha-keto acid dehydrogenase E1 component beta chain; dJ279A18.1; E1b-beta subunit of the branched-chain complex; testis secretory sperm-binding protein Li 240mP
Gene Aliases: BCKDE1B; BCKDH E1-beta; BCKDHB; E1B
UniProt ID: (Human) P21953, (Rat) P35738, (Mouse) Q6P3A8
Entrez Gene ID: (Human) 594, (Rat) 29711, (Mouse) 12040
Performance Guarantee
If an Invitrogen™ antibody doesn't perform as described on our website or datasheet,we'll replace the product at no cost to you, or provide you with a credit for a future purchase.*
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