PCCB Recombinant Mouse mAb
PCCB Recombinant Mouse mAb
- 产品详情
- 实验流程
- 背景知识
Application ![]()
| WB, IHC-P, IHC-F, IF |
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Host | Rabbit |
Clonality | Recombinant |
Physical State | Liquid |
Isotype | IgG2a, Kappa |
Purity | affinity purified by Protein G |
Buffer | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
SUBCELLULAR LOCATION | Mitochondrion matrix. |
SIMILARITY | Belongs to the AccD/PCCB family. Contains 1 carboxyltransferase domain. |
SUBUNIT | Probably a dodecamer composed of six biotin-containing alpha subunits and six beta subunits. |
DISEASE | Defects in PCCB are the cause of propionic acidemia type II (PA-2) [MIM:606054]. PA-2 is a life-threatening disease characterized by episodic vomiting, lethargy and ketosis, neutropenia, periodic thrombocytopenia, hypogammaglobulinemia, developmental retardation, and intolerance to protein. |
Important Note | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
Background Descriptions | 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] |
Dilution | WB=1:500-1:1000,IHC-P=1:50-1:100,IHC-F=,IF=0 |
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Format | 0.01M TBS(pH7.4) with 1% BSA, 0.09% (W/V) sodium azide and 50% Glyce |
Storage | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. |
Research Areas
For Research Use Only. Not For Use In Diagnostic Procedures.
Application Protocols
Provided below are standard protocols that you may find useful for product applications.
BACKGROUND
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

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