PCCA Antibody

Datasheet

Code	CSB-PA017522LA01HU
Size	US$166
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Image	Western Blot Positive WB detected in: NIH/3T3 whole cell lysate, Hela whole cell lysate All lanes: PCCA antibody at 4µg/ml Secondary Goat polyclonal to rabbit IgG at 1/50000 dilution Predicted band size: 81, 78, 76 kDa Observed band size: 81 kDa Immunohistochemistry of paraffin-embedded human liver tissue using CSB-PA017522LA01HU at dilution of 1:100 Immunohistochemistry of paraffin-embedded human heart tissue using CSB-PA017522LA01HU at dilution of 1:100
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Product Details
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Target Background

Product Details

Full Product Name

Rabbit anti-Homo sapiens (Human) PCCA Polyclonal antibody

Uniprot No.

P05165

Target Names

PCCA

Alternative Names

mitochondrial antibody; Pcca antibody; pccA COMPLEMENTATION GROUP antibody; PCCA_HUMAN antibody; PCCase subunit alpha antibody; Propanoyl CoA:carbon dioxide ligase subunit alpha antibody; Propanoyl-CoA:carbon dioxide ligase subunit alpha antibody; Propionyl CoA carboxylase alpha chain antibody; Propionyl CoA carboxylase alpha chain mitochondrial antibody; Propionyl CoA carboxylase alpha polypeptide antibody; Propionyl coenzyme A carboxylase alpha polypeptide antibody; Propionyl-CoA carboxylase alpha chain antibody

Raised in

Rabbit

Species Reactivity

Human, Mouse

Immunogen

Recombinant Human Propionyl-CoA carboxylase alpha chain, mitochondrial protein (585-720AA)

Immunogen Species

Homo sapiens (Human)

Conjugate

Non-conjugated

The PCCA Antibody (Product code: CSB-PA017522LA01HU) is Non-conjugated. For PCCA Antibody with conjugates, please check the following table.

Conjugate	Product Code	Product Name	Application
HRP	CSB-PA017522LB01HU	PCCA Antibody, HRP conjugated	ELISA
FITC	CSB-PA017522LC01HU	PCCA Antibody, FITC conjugated
Biotin	CSB-PA017522LD01HU	PCCA Antibody, Biotin conjugated	ELISA

Clonality

Polyclonal

Isotype

IgG

Purification Method

>95%, Protein G purified

Concentration

It differs from different batches. Please contact us to confirm it.

Buffer

Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4

Form

Liquid

Tested Applications

ELISA, WB, IHC

Recommended Dilution

Application	Recommended Dilution
WB	1:500-1:5000
IHC	1:20-1:200

Protocols

ELISA Protocol
Western Blotting(WB) Protocol
Immunohistochemistry (IHC) Protocol

Troubleshooting and FAQs

Antibody FAQs

Storage

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.

Lead Time

Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

Customer Reviews and Q&A

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Target Background

Function

This is one of the 2 subunits of the biotin-dependent propionyl-CoA carboxylase (PCC), a mitochondrial enzyme involved in the catabolism of odd chain fatty acids, branched-chain amino acids isoleucine, threonine, methionine, and valine and other metabolites. Propionyl-CoA carboxylase catalyzes the carboxylation of propionyl-CoA/propanoyl-CoA to D-methylmalonyl-CoA/(S)-methylmalonyl-CoA. Within the holoenzyme, the alpha subunit catalyzes the ATP-dependent carboxylation of the biotin carried by the biotin carboxyl carrier (BCC) domain, while the beta subunit then transfers the carboxyl group from carboxylated biotin to propionyl-CoA. Propionyl-CoA carboxylase also significantly acts on butyryl-CoA/butanoyl-CoA, which is converted to ethylmalonyl-CoA/(2S)-ethylmalonyl-CoA at a much lower rate. Other alternative minor substrates include (2E)-butenoyl-CoA/crotonoyl-CoA.

Gene References into Functions

The majority of patients had mutations in the PCCA gene (18/25). A total of 26 mutations were noted: 20 in the PCCA gene and 6 in PCCB gene. Seventeen mutations were novel (14 in PCCA and 3 in PCCB). The SNP c.937C>T (p.Arg313Ter), was noted in 9/36 (25%) alleles in the PCCA gene PMID: 27227689
Ten propionic acidemia mutations were confirmed, including 8 affecting the PCCA gene and 2 affecting the PCCB gene PMID: 25636094
Two PCCA mutations, c.229C-->T (p.R77W) and c.1262A-->C (p.Q421P), were identified in a PCCA-deficient patient. PMID: 24863100
Mutation analysis of the PCCA gene identified homozygous c.1284+1G>A in patient 1, c.230G>A (p.R77Q) and c.1855C>T (p.R619X) in patient 2, homozygous c.2125T>C (p.S709P) in patient 3, and only one mutant allele, c.231+1G>T in patient 4. PMID: 24464666
Thsi study presented that Propionic acidemia(PCCA mutation) associated with visual hallucinations PMID: 22156789
propionyl CoA carboxylase alpha polypeptide intronic variations causing aberrantly spliced messenger RNA is associated with propionic and methylmalonic acidemia. PMID: 17966092
cryo-electron microscopy (cryo-EM) reconstruction at 15-A resolution PMID: 20725044
pathogenicity of 11 mutations by expression studies and correlation of genotype-phenotype in PCCA-deficient propionic acidemia patients. PMID: 12385775
Data reported 9 novel PCCA gene mutations and represents an extensive update of the mutational study of propionic acidemia providing important information about the worldwide distribution of PA mutations. PMID: 12559849
analysis of propionyl-CoA carboxylase containing pathogenic mutations in the beta subunit (R165W, E168K, and R410W) and one PCCB polymorphism (A497V) and their structural and functional effects PMID: 15890657
analysis of PCCA and PCCB mutations in propionic acidemia PMID: 17051315
This work describes for the first time the high frequency of large genomic deletions in the PCCA gene, which could be due to the characteristics of the PCCA gene structure and its abundance in intronic repetitive elements. PMID: 19157943
Activities of propionyl-CoA carboxylase were not significantly different in pancreatic islets of patients with type 2 diabetes from those of the control. PMID: 19296078

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Involvement in disease

Propionic acidemia type I (PA-1)

Subcellular Location

Mitochondrion matrix.

Database Links

HGNC: 8653

OMIM: 232000

KEGG: hsa:5095

STRING: 9606.ENSP00000365462

UniGene: Hs.733632

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