Western Blot (WB)
(MCCC2 Antibody (C-term) western blot analysis in NCI-H292 cell line lysates (35ug/lane).This demonstrates the MCCC2 antibody detected the MCCC2 protein (arrow).)
Rabbit anti-Human MCCC2 Polyclonal Antibody | anti-MCCC2 antibody
MCCC2 Antibody (C-term)
Western Blot (WB)
(MCCC2 Antibody (C-term) western blot analysis in NCI-H292 cell line lysates (35ug/lane).This demonstrates the MCCC2 antibody detected the MCCC2 protein (arrow).)
Western Blot (WB)
(MCCC2 Antibody (C-term) western blot analysis in NCI-H292 cell line lysates (35ug/lane).This demonstrates the MCCC2 antibody detected the MCCC2 protein (arrow).)
NCBI and Uniprot Product Information
NCBI Description
This gene encodes the small subunit of 3-methylcrotonyl-CoA carboxylase. This enzyme functions as a heterodimer and catalyzes the carboxylation of 3-methylcrotonyl-CoA to form 3-methylglutaconyl-CoA. Mutations in this gene are associated with 3-Methylcrotonylglycinuria, an autosomal recessive disorder of leucine catabolism. [provided by RefSeq, Jul 2008]
Uniprot Description
MCCC2: Defects in MCCC2 are the cause of methylcrotonoyl-CoA carboxylase 2 deficiency (MCC2D). An autosomal recessive disorder of leucine catabolism. The phenotype is variable, ranging from neonatal onset with severe neurological involvement to asymptomatic adults. There is a characteristic organic aciduria with massive excretion of 3-hydroxyisovaleric acid and 3-methylcrotonylglycine, usually in combination with a severe secondary carnitine deficiency. Belongs to the AccD/PCCB family. 2 isoforms of the human protein are produced by alternative splicing.
Protein type: Ligase; Mitochondrial; Amino Acid Metabolism - valine, leucine and isoleucine degradation; EC 6.4.1.4
Chromosomal Location of Human Ortholog: 5q12-q13
Cellular Component: mitochondrion; mitochondrial matrix; cytosol
Molecular Function: protein binding; methylcrotonoyl-CoA carboxylase activity; ATP binding
Biological Process: coenzyme A metabolic process; vitamin metabolic process; leucine catabolic process; branched chain family amino acid catabolic process; water-soluble vitamin metabolic process; biotin metabolic process
Disease: 3-methylcrotonyl-coa Carboxylase 2 Deficiency
Research Articles on MCCC2
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Product Notes
The MCCC2 mccc2 (Catalog #AAA9211665) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The immunogen sequence is 468-496. The MCCC2 Antibody (C-term) reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's MCCC2 can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB), ELISA (EIA). WB~~1:1000. Researchers should empirically determine the suitability of the MCCC2 mccc2 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "MCCC2, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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