catalog number :
MBS9211639
products full name :
BRP44L Antibody (C-term)
products short name :
[BRP44L]
products name syn :
[Mitochondrial pyruvate carrier 1; Brain protein 44-like protein; MPC1; BRP44L]
other names :
[mitochondrial pyruvate carrier 1 isoform 1; Mitochondrial pyruvate carrier 1; mitochondrial pyruvate carrier 1; mitochondrial pyruvate carrier 1; Brain protein 44-like protein]
products gene name :
[MPC1]
other gene names :
[MPC1; MPC1; MPYCD; BRP44L; CGI-129; dJ68L15.3; BRP44L]
uniprot entry name :
MPC1_HUMAN
specificity :
This BRP44L antibody is generated from a rabbit immunized with a KLH conjugated synthetic peptide between 96-129 amino acids from the C-terminal region of human BRP44L.
purity :
Purified Rabbit Polyclonal Antibody (Pab)
form :
Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.
concentration :
Vial Concentration: 0.473
storage stability :
Maintain refrigerated at 2-8 degree C for up to 2 weeks. For long term storage store at -20 degree C in small aliquots to prevent freeze-thaw cycles.
tested application :
Western Blot (WB), ELISA (EIA)
image1 heading :
Western Blot (WB)
other info1 :
Crown Antibody: Yes
other info2 :
Antigen Source: HUMAN. Cellular Location: Mitochondrion inner membrane; Multi-pass membrane protein
products categories :
Crown Antibodies; Cell Biology
products description :
Mediates the uptake of pyruvate into mitochondria.
products references :
Zhang Q.-H.,et al.Genome Res. 10:1546-1560(2000). Lai C.-H.,et al.Genome Res. 10:703-713(2000). Ota T.,et al.Nat. Genet. 36:40-45(2004). Mungall A.J.,et al.Nature 425:805-811(2003). Yu W.-Q.,et al.Submitted (JUN-2000) to the EMBL/GenBank/DDBJ databases.
ncbi acc num :
NP_057182.1
ncbi gb acc num :
NM_016098.3
ncbi summary :
The protein encoded by this gene is part of an MPC1/MPC2 heterodimer that is responsible for transporting pyruvate into mitochondria. The encoded protein is found in the inner mitochondrial membrane. Defects in this gene are a cause of mitochondrial pyruvate carrier deficiency. Several transcript variants, some protein coding and one non-protein coding, have been found for this gene. [provided by RefSeq, Aug 2012]
uniprot summary :
BRP44L: Mediates the uptake of pyruvate into mitochondria. Defects in MPC1 are the cause of mitochondrial pyruvate carrier deficiency (MPYCD). An autosomal recessive metabolic disorder characterized by severely delayed psychomotor development, mild dysmorphic features, hepatomegaly, marked metabolic acidosis, hyperlactacidemia with normal lactate/pyruvate, and encephalopathy. Some patients have epilepsy and peripheral neuropathy. Belongs to the mitochondrial pyruvate carrier (MPC) family. Protein type: Membrane protein, multi-pass; Mitochondrial. Chromosomal Location of Human Ortholog: 6q27. Cellular Component: mitochondrion; mitochondrial inner membrane; integral to membrane. Molecular Function: pyruvate transmembrane transporter activity. Biological Process: cellular metabolic process; mitochondrial pyruvate transport; pyruvate metabolic process. Disease: Mitochondrial Pyruvate Carrier Deficiency