KO Validated Antibodies 1

Anti-PCCB Antibody (CAB18072)[KO Validated]

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SKU:
CAB18072
Product Type:
Antibody
Reactivity:
Human
Reactivity:
Mouse
Host Species:
Rabbit
Isotype:
IgG
Research Area:
Cell Biology

Description

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Antibody Name:Anti-PCCB Antibody [KO Validated]
Antibody SKU:CAB18072
Antibody Size:20uL, 50uL, 100uL
Application:WB IF
Reactivity:Human, Mouse
Host Species:Rabbit
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 260-539 of human PCCB (NP_000523.2).
Application:WB IF
Recommended Dilution:WB 1:500 - 1:2000 IF 1:50 - 1:200
Reactivity:Human, Mouse
Positive Samples:293T
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 260-539 of human PCCB (NP_000523.2).
Purification Method:Affinity purification
Storage Buffer:Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:IgG
Sequence:AFEN DVDA LCNL RDFF NYLP LSSQ DPAP VREC HDPS DRLV PELD TIVP LEST KAYN MVDI IHSV VDER EFFE IMPN YAKN IIVG FARM NGRT VGIV GNQP KVAS GCLD INSS VKGA RFVR FCDA FNIP LITF VDVP GFLP GTAQ EYGG IIRH GAKL LYAF AEAT VPKV TVIT RKAY GGAY DVMS SKHL CGDT NYAW PTAE IAVM GAKG AVEI IFKG HENV EAAQ AEYI EKFA NPFP AAVR GFVD DIIQ PSST RARI CCDL DVLA SKKV QRPW RKHA NIPL
Gene ID:5096
Uniprot:P05166
Cellular Location:Mitochondrion matrix
Calculated MW:58kDa/60kDa
Observed MW:50kDa
Anti-PCCB Antibody [KO Validated] (CAB18072)Western blot analysis of extracts from normal (control) and PCCB knockout (KO) 293T cells, using PCCB antibody (CAB18072) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 10s.
Synonyms:PCCB
Background:The protein encoded by this gene is a subunit of the propionyl-CoA carboxylase (PCC) enzyme, which is involved in the catabolism of propionyl-CoA. PCC is a mitochondrial enzyme that probably acts as a dodecamer of six alpha subunits and six beta subunits. This gene encodes the beta subunit of PCC. Defects in this gene are a cause of propionic acidemia type II (PA-2). Multiple transcript variants encoding different isoforms have been found for this gene.
UniProt Protein Function:PCCB: Defects in PCCB are the cause of propionic acidemia type II (PA-2). PA-2 is a life-threatening disease characterized by episodic vomiting, lethargy and ketosis, neutropenia, periodic thrombocytopenia, hypogammaglobulinemia, developmental retardation, and intolerance to protein. Belongs to the AccD/PCCB family. 2 isoforms of the human protein are produced by alternative splicing.
UniProt Protein Details:

Protein type:EC 6.4.1.3; Ligase; Carbohydrate Metabolism - propanoate; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Mitochondrial

Chromosomal Location of Human Ortholog: 3q21-q22

Cellular Component: mitochondrion; mitochondrial matrix; cytosol

Molecular Function:propionyl-CoA carboxylase activity; ATP binding

Biological Process: vitamin metabolic process; fatty acid beta-oxidation; short-chain fatty acid catabolic process; cellular lipid metabolic process; biotin metabolic process; water-soluble vitamin metabolic process

Disease: Propionic Acidemia

NCBI Summary:The protein encoded by this gene is a subunit of the propionyl-CoA carboxylase (PCC) enzyme, which is involved in the catabolism of propionyl-CoA. PCC is a mitochondrial enzyme that probably acts as a dodecamer of six alpha subunits and six beta subunits. This gene encodes the beta subunit of PCC. Defects in this gene are a cause of propionic acidemia type II (PA-2). Multiple transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, May 2010]
UniProt Code:P05166
NCBI GenInfo Identifier:124106304
NCBI Gene ID:5096
NCBI Accession:P05166.3
UniProt Secondary Accession:P05166,Q16813, Q96CX0, B7Z2Z4,
UniProt Related Accession:P05166
Molecular Weight:60,521 Da
NCBI Full Name:Propionyl-CoA carboxylase beta chain, mitochondrial
NCBI Synonym Full Names:propionyl CoA carboxylase, beta polypeptide
NCBI Official Symbol:PCCB  
NCBI Protein Information:propionyl-CoA carboxylase beta chain, mitochondrial; PCCase subunit beta; propanoyl-CoA:carbon dioxide ligase subunit beta; propionyl Coenzyme A carboxylase, beta polypeptide
UniProt Protein Name:Propionyl-CoA carboxylase beta chain, mitochondrial
UniProt Synonym Protein Names:Propanoyl-CoA:carbon dioxide ligase subunit beta
Protein Family:Phycoerythrocyanin
UniProt Gene Name:PCCB  
UniProt Entry Name:PCCB_HUMAN
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