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Invitrogen
BCKDHB Polyclonal Antibody
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FIGURE: 1 / 1
BCKDHB Antibody (PA5-87963) in WB
Western blot analysis of BCKDHB in various lysates. Samples were incubated with BCKDHB Polyclonal antibody (Product # PA5-87963) using a dilution of 1:2,000, followed by HRP Goat Anti-Rabbit IgG (H+L) at a dilution of 1:10,000. Lysates/proteins: 25 µg per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit. Exposure time: 60s.
Product Details
PA5-87963
Applications
Tested Dilution
Publications
Product Specifications
Species Reactivity
Mouse,
Rat
Host/Isotype
Rabbit
/ IgG
Class
Polyclonal
Type
Antibody
Immunogen
Recombinant fusion protein containing a sequence corresponding to amino acids 183-392 of human BCKDHB (NP_8988711)
Conjugate
Form
Liquid
Concentration
2.7 mg/mL
Purification
Affinity Chromatography
Storage buffer
PBS, pH 7.3, with 50% glycerol
Contains
0.02% sodium azide
Storage conditions
-20°C, Avoid Freeze/Thaw Cycles
Shipping conditions
Wet ice
RRID
AB_2804545
Product Specific Information
Immunogen sequence: RSPWGCVGHG ALYHSQSPEA FFAHCPGIKV VIPRSPFQAK GLLLSCIEDK NPCIFFEPKI LYRAAAEEVP IEPYNIPLSQ AEVIQEGSDV TLVAWGTQVH VIREVASMAK EKLGVSCEVI DLRTIIPWDV DTICKSVIKT GRLLISHEAP LTGGFASEIS STVQEECFLN LEAPISRVCG YDTPFPHIFE PFYIPDKWKC YDALRKMINY; Positive Samples: Mouse pancreas; Cellular Location: Mitochondrion matrix
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.
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Bioinformatics
Protein Aliases: 2-oxoisovalerate dehydrogenase beta subunit; 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial; BCKD BCKDH E1-beta; BCKDE1B; BCKDH E1-beta; branched chain keto acid dehydrogenase E1 beta; Branched-chain alpha-keto acid dehydrogenase E1 component beta chain; dJ279A18.1
Gene Aliases: BCKDE1B; BCKDH E1-beta; Bckdhb
UniProt ID: (Rat) P35738, (Mouse) Q6P3A8
Entrez Gene ID: (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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