Anti-PCCA Antibody (CAB9486)
- SKU:
- CAB9486
- Product type:
- Antibody
- Application:
- WB
- Reactivity:
- Human
- Mouse
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Research Area:
- Metabolism
Frequently bought together:
Description
| Antibody Name: | Anti-PCCA Antibody |
| Antibody SKU: | CAB9486 |
| Antibody Size: | 20uL, 50uL, 100uL |
| Application: | WB |
| Reactivity: | Mouse, Rat |
| Host Species: | Rabbit |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 449-728 of human PCCA (NP_000273.2). |
| Application: | WB |
| Recommended Dilution: | WB 1:1000 - 1:3000 |
| Reactivity: | Mouse, Rat |
| Positive Samples: | Mouse pancreas, Mouse liver, Mouse brain, Mouse kidney, Rat kidney |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 449-728 of human PCCA (NP_000273.2). |
| 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: | ISKL ITYG SDRT EALK RMAD ALDN YVIR GVTH NIAL LREV IINS RFVK GDIS TKFL SDVY PDGF KGHM LTKS EKNQ LLAI ASSL FVAF QLRA QHFQ ENSR MPVI KPDI ANWE LSVK LHDK VHTV VASN NGSV FSVE VDGS KLNV TSTW NLAS PLLS VSVD GTQR TVQC LSRE AGGN MSIQ FLGT VYKV NILT RLAA ELNK FMLE KVTE DTSS VLRS PMPG VVVA VSVK PGDA VAEG QEIC VIEA MKMQ NSMT AGKT GTVK SVHC QAGD TVGE GDLL VELE |
| Gene ID: | 5095 |
| Uniprot: | P05165 |
| Cellular Location: | Mitochondrion matrix |
| Calculated MW: | 75kDa/77kDa/80kDa |
| Observed MW: | 80kDa |
| Synonyms: | PCCA |
| Background: | The protein encoded by this gene is the alpha subunit of the heterodimeric mitochondrial enzyme Propionyl-CoA carboxylase. PCCA encodes the biotin-binding region of this enzyme. Mutations in either PCCA or PCCB (encoding the beta subunit) lead to an enzyme deficiency resulting in propionic acidemia. Multiple transcript variants encoding different isoforms have been found for this gene. |
| UniProt Protein Function: | PCCA: Defects in PCCA are the cause of propionic acidemia type I (PA-1). PA-1 is a life-threatening disease characterized by episodic vomiting, lethargy and ketosis, neutropenia, periodic thrombocytopenia, hypogammaglobulinemia, developmental retardation, and intolerance to protein. 2 isoforms of the human protein are produced by alternative splicing. |
| UniProt Protein Details: | Protein type:Carbohydrate Metabolism - propanoate; Ligase; Mitochondrial; EC 6.4.1.3; Amino Acid Metabolism - valine, leucine and isoleucine degradation Chromosomal Location of Human Ortholog: 13q32 Cellular Component: cytosol; mitochondrial matrix Molecular Function:biotin binding; enzyme binding Biological Process: biotin metabolic process; short-chain fatty acid catabolic process Disease: Propionic Acidemia |
| NCBI Summary: | The protein encoded by this gene is the alpha subunit of the heterodimeric mitochondrial enzyme Propionyl-CoA carboxylase. PCCA encodes the biotin-binding region of this enzyme. Mutations in either PCCA or PCCB (encoding the beta subunit) lead to an enzyme deficiency resulting in propionic acidemia. Multiple transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, May 2010] |
| UniProt Code: | P05165 |
| NCBI GenInfo Identifier: | 308153661 |
| NCBI Gene ID: | 5095 |
| NCBI Accession: | P05165.4 |
| UniProt Secondary Accession: | P05165,Q15979, Q8WXQ7, B4DKY8, B4DPF9, C9JPQ8, |
| UniProt Related Accession: | P05165 |
| Molecular Weight: | 75,002 Da |
| NCBI Full Name: | Propionyl-CoA carboxylase alpha chain, mitochondrial |
| NCBI Synonym Full Names: | propionyl-CoA carboxylase alpha subunit |
| NCBI Official Symbol: | PCCA |
| NCBI Protein Information: | propionyl-CoA carboxylase alpha chain, mitochondrial |
| UniProt Protein Name: | Propionyl-CoA carboxylase alpha chain, mitochondrial |
| UniProt Synonym Protein Names: | Propanoyl-CoA:carbon dioxide ligase subunit alpha |
| UniProt Gene Name: | PCCA |
| UniProt Entry Name: | PCCA_HUMAN |
 | Western blot analysis of extracts of various cell lines, using PCCA antibody (CAB9486) at 1:3000 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: 1s. |
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