Rabbit Anti-MCCC1 Polyclonal Antibody – Cat# MBS1758815

Figure 1. Western blot analysis of MCCC1 using anti-MCCC1 antibody (MBS1758815).
Electrophoresis was performed on a 5-20% SDS-PAGE gel at 70V (Stacking gel)/90V (Resolving gel) for 2-3 hours. The sample well of each lane was loaded with 30 ug of sample under reducing conditions.
Lane 1: human SIHA whole cell lysates,
Lane 2: human RT4 whole cell lysates,
Lane 3: rat liver tissue lysates.
After electrophoresis, proteins were transferred to a nitrocellulose membrane at 150 mA for 50-90 minutes. Blocked the membrane with 5% non-fat milk/TBS for 1.5 hour at RT. The membrane was incubated with rabbit anti-MCCC1 antigen affinity purified polyclonal antibody (#MBS1758815) at 0.5ug/mL overnight at 4 degree C, then washed with TBS-0.1%Tween 3 times with 5 minutes each and probed with a goat anti-rabbit IgG-HRP secondary antibody at a dilution of 1:5000 for 1.5 hour at RT. The signal is developed using an Enhanced Chemiluminescent detection (ECL) kit (#MBS176460) with Tanon 5200 system. A specific band was detected for MCCC1 at approximately 75 kDa. The expected band size for MCCC1 is at 80 kDa.

MCCC1 Antibody – Rabbit Polyclonal

Name: MCCC1 Antibody; Polyclonal; Rabbit; Human/Mouse/Rat-Reactive; MyBioSource
Brand: MyBioSource
SKU: MBS1758815
Price: 210 USD
Availability: InStock
Rating: 5 (10 reviews)

For Research Use Only. Not for use in diagnostic procedures. Catalog #: MBS1758815

The brand Picoband indicates this is a premium antibody that guarantees superior quality, high affinity, and strong signals with minimal background in Western blot applications. Only our best-performing antibodies are designated as Picoband, ensuring… More details

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Product Documentation

Sample Manual Insert View PDF

Testing Data View PDF

Certificate of Analysis (COA) View PDF

QC Report View PDF

Material Safety Data Sheet View PDF

Technical Datasheet View PDF

Protocol View PDF

Product Overview

Online Datasheet in PDF

Product Name

MCCC1, Polyclonal Antibody

Full Product Name

Anti-MCCC1 Antibody Picoband

Product Gene Name

anti-MCCC1 antibody

Product Synonym Names

BAG family molecular chaperone regulator 5; BAG family molecular chaperone regulator 5; BAG family molecular chaperone regulator 5; BAG-5; Bcl-2-associated athanogene 5; BAG5; KIAA0873

Clonality

Polyclonal

Isotype

Rabbit IgG

Host

Rabbit

Species Reactivity

Human, Mouse, Rat

Form/Format

Lyophilized. Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.

Purity/Purification

Immunogen affinity purified.

Specificity

Expressed in all tissues examined with lower levels in brain and testis.

Preparation and Storage

At -20 degree C for one year from date of receipt. After reconstitution, at 4 degree C for one month. It can also be aliquotted and stored frozen at -20 degree C for six months.Avoid repeated freezing and thawing.

Molecular Weight

80,473 Da

Chromosome Location

Chromosome: 3; NC_000003.11 (182733006..182817373, complement). Location: 3q27

OMIM

210200

3D Structure

ModBase 3D Structure for Q96RQ3

Immunogen

E coli-derived human MCCC1 recombinant protein (Position: Q104-E715).

Subcellular Localization

Perinuclear region

Reconstitution

Adding 0.2 ml of distilled water will yield a concentration of 500ug/ml.

Cross Reactivity

No cross-reactivity with other proteins.

Protein Function

Inhibits both auto-ubiquitination of PARK2 and ubiquitination of target proteins by PARK2 (By similarity). May function as a nucleotide exchange factor for HSP/HSP70, promoting ADP release, and activating Hsp70-mediated refolding.

Product Categories/Family for anti-MCCC1 antibody

Primary Antibodies; Interleukins

ISO Certification

Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.

Applications Tested/Suitable for anti-MCCC1 antibody

Western Blot (WB), Immunohistochemistry (IHC), Immunocytochemistry (ICC), Immunofluorescence (IF), ELISA (EIA)

Application Notes for anti-MCCC1 antibody

WB: 0.25-0.5ug/ml, Human, Rat
IHC: 2-5ug/ml, Human, Mouse, Rat
ICC/IF: 5ug/ml, Human
ELISA: 0.1-0.5ug/ml

Product References and Citations for anti-MCCC1 antibody

1. Baumgartner, M. R., Almashanu, S., Suormala, T., Obie, C., Cole, R. N., Packman, S., Baumgartner, E. R., Valle, D. The molecular basis of human 3-methylcrotonyl-CoA carboxylase deficiency. J. Clin. Invest. 107: 495-504, 2001.2. Baumgartner, M. R., Dantas, M. F., Suormala, T., Almashanu, S., Giunta, C., Friebel, D., Gebhardt, B., Fowler, B., Hoffmann, G. F., Baumgartner, E. R., Valle, D. Isolated 3-methylcrotonyl-CoA carboxylase deficiency: evidence for an allele-specific dominant negative effect and responsiveness to biotin therapy. Am. J. Hum. Genet. 75: 790-800, 2004.3. Gallardo, M. E., Desviat, L. R., Rodriguez, J. M., Esparza-Gordillo, J., Perez-Cerda, C., Perez, B., Rodriguez-Pombo, P., Criado, O., Sanz, R., Morton, D. H., Gibson, K. M., Le, T. P., Ribes, A., Rodriguez de Cordoba, S., Ugarte, M., Penalva, M. A. The molecular basis of 3-methylcrotonylglycinuria, a disorder of leucine catabolism. Am. J. Hum. Genet. 68: 334-346, 2001.

Other Notes

Small volumes of anti-MCCC1 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Searchable Terms for MCCC1 purchase

MBS1758815 is an antibody targeting MCCC1. Also known as Anti-MCCC1 Antibody Picoband. Reactive in Human, Mouse, Rat. Host: Rabbit.

Additional Details

NCBI/Uniprot data below describe general gene information for MCCC1. It may not necessarily be applicable to this product.

NCBI GI #

116805327

NCBI GeneID

56922

NCBI Accession #

NP_064551.3

UniProt Primary Accession #

Q96RQ3

UniProt Secondary Accession #

Q59ES4; Q9H959; Q9NS97

UniProt Related Accession #

Q96RQ3

GenBank Nucleotide #

NM_020166.3

NCBI Official Full Name

methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial

NCBI Official Synonym Full Names

methylcrotonoyl-CoA carboxylase 1 (alpha)

NCBI Official Symbol

MCCC1

NCBI Official Synonym Symbols

MCCA; MCC-B

UniProt Protein Name

Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial

UniProt Synonym Protein Names

3-methylcrotonyl-CoA carboxylase 1; 3-methylcrotonyl-CoA carboxylase biotin-containing subunit; 3-methylcrotonyl-CoA:carbon dioxide ligase subunit alpha

UniProt Gene Name

MCCC1

UniProt Synonym Gene Names

MCCA; MCCase subunit alpha

UniProt Entry Name

MCCA_HUMAN

NCBI Protein Information

methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial; MCCase subunit alpha; 3-methylcrotonyl-CoA carboxylase 1; methylcrotonoyl-Coenzyme A carboxylase 1 (alpha); 3-methylcrotonyl-CoA:carbon dioxide ligase subunit alpha; 3-methylcrotonyl-CoA carbox

NCBI Summary for MCCC1

This gene encodes the large 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 Comments for MCCC1

Function: Biotin-attachment subunit of the 3-methylcrotonyl-CoA carboxylase, an enzyme that catalyzes the conversion of 3-methylcrotonyl-CoA to 3-methylglutaconyl-CoA, a critical step for leucine and isovaleric acid catabolism. Ref.9

Catalytic activity: ATP + 3-methylcrotonoyl-CoA + HCO3- = ADP + phosphate + 3-methylglutaconyl-CoA. Ref.9

Cofactor: Biotin.

Pathway: Amino-acid degradation; L-leucine degradation; (S)-3-hydroxy-3-methylglutaryl-CoA from 3-isovaleryl-CoA: step 2/3.

Subunit structure: Probably a dodecamer composed of six biotin-containing alpha subunits (MCCC1) and six beta (MCCC2) subunits.

Subcellular location: Mitochondrion matrix Ref.8.

Involvement in disease: Methylcrotonoyl-CoA carboxylase 1 deficiency (MCC1D) [MIM:210200]: 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.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.1 Ref.3 Ref.4 Ref.12

Sequence similarities: Contains 1 ATP-grasp domain.Contains 1 biotin carboxylation domain.Contains 1 biotinyl-binding domain.

Sequence caution: The sequence BAD92974.1 differs from that shown. Reason: Erroneous initiation.

Research Articles on MCCC1

1. 3-methylcrotonyl-CoA carboxylase inhibition has a role in increased excretion of 3-hydroxyisovaleric acid in valproate-treated patients

Precautions

All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.

Disclaimer

While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.

Product Note

Select online data sheet information is drawn from bioinformatics databases, occasionally resulting in ambiguous or non-relevant product information. It is the responsibility of the customer to review, verify, and evaluate the information to make sure it matches their requirements before purchasing the kit. Our ELISA Kit assays are dynamic research tools and sometimes they may be updated and improved. If the format of this assay is important to you then please request the current manual or contact our technical support team with a presales inquiry before placing an order. We will confirm the current details of the assay. We cannot guarantee the sample manual posted online is the most current manual, it is intended to serve as an example only. Please refer to the instructions For Use provided with the assay kit for precise details.

Product PubMed Publications

Products associated with anti-MCCC1 antibody

Reference Product	PubMed Publications
MCCC2 antibody	>5 publications with MCCC1 and MCCC2

Pathways associated with anti-MCCC1 antibody

Products by Pathway	Pathway Diagram
Biotin Transport And Metabolism Pathway antibodies	Biotin Transport And Metabolism Pathway Diagram
Branched-chain Amino Acid Catabolism Pathway antibodies	Branched-chain Amino Acid Catabolism Pathway Diagram
Leucine Degradation, Leucine => Acetoacetate + Acetyl-CoA Pathway antibodies	Leucine Degradation, Leucine => Acetoacetate + Acetyl-CoA Pathway Diagram
Leucine Degradation, Leucine => Acetoacetate + Acetyl-CoA Pathway antibodies	Leucine Degradation, Leucine => Acetoacetate + Acetyl-CoA Pathway Diagram
Metabolism Pathway antibodies	Metabolism Pathway Diagram
Metabolism Of Amino Acids And Derivatives Pathway antibodies	Metabolism Of Amino Acids And Derivatives Pathway Diagram
Metabolism Of Vitamins And Cofactors Pathway antibodies	Metabolism Of Vitamins And Cofactors Pathway Diagram
Metabolism Of Water-soluble Vitamins And Cofactors Pathway antibodies	Metabolism Of Water-soluble Vitamins And Cofactors Pathway Diagram
Valine, Leucine And Isoleucine Degradation Pathway antibodies	Valine, Leucine And Isoleucine Degradation Pathway Diagram
Valine, Leucine And Isoleucine Degradation Pathway antibodies	Valine, Leucine And Isoleucine Degradation Pathway Diagram

Diseases associated with anti-MCCC1 antibody

Disease Name	Pubmed Publications
3-methylcrotonyl CoA carboxylase 1 deficiency Antibodies	>3 publications with MCCC1 and 3-methylcrotonyl CoA carboxylase 1 deficiency
Death Antibodies	>2 publications with MCCC1 and Death
Alzheimer Disease Antibodies	>1 publications with MCCC1 and Alzheimer Disease

Organs/Tissues associated with anti-MCCC1 antibody

Organ/Tissue Name	Pubmed Publications
Brain Antibodies	>2 publications with MCCC1 and Brain
Spleen Antibodies	>1 publications with MCCC1 and Spleen
Ovary Antibodies	>1 publications with MCCC1 and Ovary
Heart Antibodies	>1 publications with MCCC1 and Heart
Muscle Antibodies	>1 publications with MCCC1 and Muscle
Skin Antibodies	>1 publications with MCCC1 and Skin
Blood Antibodies	>1 publications with MCCC1 and Blood
Liver Antibodies	>1 publications with MCCC1 and Liver