BCKDHB (NM_183050) Human Mass Spec Standard

Specifications

Product Data
Description	BCKDHB MS Standard C13 and N15-labeled recombinant protein (NP_898871)
Species	Human
Expression Host	HEK293
Expression cDNA Clone or AA Sequence	RC208059
Predicted MW	43.1 kDa
Protein Sequence	Sequence Link (showhide) >RC208059 protein sequence Red=Cloning site Green=Tags(s) MAVVAAAAGWLLRLRAAGAEGHWRRLPGAGLARGFLHPAATVEDAAQRRQVAHFTFQPDPEPREYGQTQK MNLFQSVTSALDNSLAKDPTAVIFGEDVAFGGVFRCTVGLRDKYGKDRVFNTPLCEQGIVGFGIGIAVTG ATAIAEIQFADYIFPAFDQIVNEAAKYRYRSGDLFNCGSLTIRSPWGCVGHGALYHSQSPEAFFAHCPGI KVVIPRSPFQAKGLLLSCIEDKNPCIFFEPKILYRAAAEEVPIEPYNIPLSQAEVIQEGSDVTLVAWGTQ VHVIREVASMAKEKLGVSCEVIDLRTIIPWDVDTICKSVIKTGRLLISHEAPLTGGFASEISSTVQEECF LNLEAPISRVCGYDTPFPHIFEPFYIPDKWKCYDALRKMINY TRTRPLEQKLISEEDLAANDILDYKDDDDKV
Tag	C-Myc/DDK
Purity	> 80% as determined by SDS-PAGE and Coomassie blue staining
Concentration	>0.05 µg/µL as determined by microplate BCA method
Labeling Method	Labeled with [U- 13C6, 15N4]-L-Arginine and [U- 13C6, 15N2]-L-Lysine
Buffer	25 mM Tris-HCl, 100 mM glycine, pH 7.3
Reference Data
RefSeq	NP_898871
RefSeq Size	3712
RefSeq ORF	1176
Synonyms	BCKDE1B; BCKDH E1-beta; E1B
Locus ID	594
Cytogenetics	6q14.1
Summary	This gene encodes the E1 beta subunit of branched-chain keto acid dehydrogenase, which is a multienzyme complex associated with the inner membrane of mitochondria. This enzyme complex functions in the catabolism of branched-chain amino acids. Mutations in this gene 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. Alternative splicing at this locus results in multiple transcript variants. [provided by RefSeq, Jan 2016]
Protein Pathways	Metabolic pathways, Valine, leucine and isoleucine degradation

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