JavaScript is disabled in your browser. Please enable JavaScript to view this website.
AB197282

Anti-MCCC1 antibody

0

(1 Review)

|

(1 Publication)

Rabbit Polyclonal MCCC1 antibody. Suitable for WB, IHC-P and reacts with Human, Mouse samples. Cited in 1 publication. Immunogen corresponding to Recombinant Fragment Protein within Human MCCC1 aa 500-700.

View Alternative Names

MCCA, MCCC1, MCCase subunit alpha, 3-methylcrotonyl-CoA carboxylase 1, 3-methylcrotonyl-CoA carboxylase biotin-containing subunit, 3-methylcrotonyl-CoA:carbon dioxide ligase subunit alpha

2 Images
Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-MCCC1 antibody (AB197282)
  • IHC-P

Supplier Data

Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-MCCC1 antibody (AB197282)

Immunohistochemical analysis of formalin-fixed paraffin-embedded human fetal liver tissue labeling MCCC1 with ab197282 at a 1/100 dilution.

Western blot - Anti-MCCC1 antibody (AB197282)
  • WB

Supplier Data

Western blot - Anti-MCCC1 antibody (AB197282)

All lanes:

Western blot - Anti-MCCC1 antibody (ab197282) at 1/500 dilution

Lane 1:

293 cell lysate

Lane 2:

Mouse heart lysate

Predicted band size: 80 kDa

false

Key facts

Host species

Rabbit

Clonality

Polyclonal

Isotype

IgG

Carrier free

No

Reacts with

Mouse, Human

Applications

WB, IHC-P

applications

Immunogen

Recombinant Fragment Protein within Human MCCC1 aa 500-700. The exact immunogen used to generate this antibody is proprietary information.

Q96RQ3

style
left-column

Reactivity data

{ "title": "Reactivity Data", "filters": { "stats": ["", "Species", "Dilution Info", "Notes"], "tabs": { "all-applications": {"fullname" : "All Applications", "shortname": "All Applications"}, "WB" : {"fullname" : "Western blot", "shortname":"WB"}, "IHCP" : {"fullname" : "Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections)", "shortname":"IHC-P"} }, "product-promise": { "all": "all", "testedAndGuaranteed": "tested", "guaranteed": "expected", "predicted": "predicted", "notRecommended": "not-recommended" } }, "values": { "Human": { "WB-species-checked": "testedAndGuaranteed", "WB-species-dilution-info": "1/200 - 1/1000", "WB-species-notes": "<p></p>", "IHCP-species-checked": "testedAndGuaranteed", "IHCP-species-dilution-info": "1/100 - 1/500", "IHCP-species-notes": "<p></p>" }, "Mouse": { "WB-species-checked": "testedAndGuaranteed", "WB-species-dilution-info": "1/200 - 1/1000", "WB-species-notes": "<p></p>", "IHCP-species-checked": "guaranteed", "IHCP-species-dilution-info": "", "IHCP-species-notes": "" } } }
style
left-column
style
left-column

Properties and storage information

Form
Lyophilized
Reconstitution
reconstitute with water at 200µL
Purification technique
Affinity purification Immunogen
Storage buffer
pH: 7.2 Preservative: 0.02% Sodium azide Constituents: PBS, 1% BSA
Shipped at conditions
Blue Ice
Appropriate short-term storage duration
1-2 weeks
Appropriate short-term storage conditions
+4°C
Appropriate long-term storage conditions
-20°C
Aliquoting information
Upon delivery aliquot
Storage information
Avoid freeze / thaw cycle
style
left-column

Supplementary information

This supplementary information is collated from multiple sources and compiled automatically.

The target MCCC1 also known as 3-methylcrotonyl-CoA carboxylase 1 is an enzyme with a molecular mass of approximately 83 kDa. It functions as one component of the mitochondrial protein complex that catalyzes the carboxylation of 3-methylcrotonyl-CoA to 3-methylglutaconyl-CoA an important step in the leucine catabolic pathway. MCCC1 expresses ubiquitously in human tissues with significant expression levels detected in the liver heart and muscle tissues highlighting its metabolic importance.
Biological function summary

MCCC1 operates as a subunit of the larger 3-methylcrotonyl-CoA carboxylase complex a mitochondrial enzyme important to leucine degradation. By facilitating an early step in this amino acid's breakdown it plays a vital part in the metabolic processes that produce energy and carbon skeletons for biosynthetic pathways. MCCC1's activity reflects its responsibility in coordinating with biotin as a cofactor within the complex ensuring effective enzyme performance.

Pathways

MCCC1 participates in the leucine catabolism and branched-chain amino acid degradation pathways. These pathways incorporate proteins such as the HMG-CoA lyase and enoyl-CoA hydratase which work together to complete the degradation of leucine to acetoacetate and other intermediates. MCCC1 ensures the flow of substrate through these pathways creating links among several metabolic processes important for maintaining energy homeostasis and mitochondrial function.

MCCC1 mutations lead to 3-methylcrotonyl-CoA carboxylase deficiency a metabolic disorder characterized by an inability to properly break down leucine causing symptoms like hypoglycemia developmental delays and muscle weakness. Connections to this disorder reveal interrelations with biotinidase as insufficient biotin impacts enzymatic function involving biotin-dependent carboxylases including MCCC1. Exploring these relationships furthers understanding of MCCC1's role in metabolic homeostasis and mitochondrial energy production.
style
left-column
style
left-column

Product protocols

For this product, it's our understanding that no specific protocols are required. You can visit:
style
left-column

Target data

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.
See full target information MCCC1
style
left-column

Publications (1)

Recent publications for all applications. Explore the full list and refine your search

The Journal of biological chemistry 292:4499-4518 PubMed28100784

2017

Mitochondrial -GlcNAc Transferase (mOGT) Regulates Mitochondrial Structure, Function, and Survival in HeLa Cells.

Applications

Unspecified application

Species

Unspecified reactive species

Juliana L Sacoman,Raul Y Dagda,Amanda R Burnham-Marusich,Ruben K Dagda,Patricia M Berninsone
View all publications
style
left-column
style
left-column
style
right-column

Complementary Products

Primary Antibodies

AB125920

Anti-MCC antibody

Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-MCC antibody (AB125920)

  • 0
  • 0
Proteins & Peptides

AB163430

Recombinant Human MCCC1 protein

SDS-PAGE - Recombinant Human MCCC1 protein (AB163430)

  • 0
  • 0
Primary Antibodies

AB109235

Anti-Xanthine Oxidase antibody [EPR4605]

RabMAb|Recombinant|20ul selling size

Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Xanthine Oxidase antibody [EPR4605] (AB109235)

  • 4
  • 3
Primary Antibodies

AB96171

Anti-VNN1/Vanin-1 antibody

Western blot - Anti-VNN1/Vanin-1 antibody (AB96171)

  • 0
  • 0

Product promise

We are committed to supporting your work with high-quality reagents, and we're here for you every step of the way. In the unlikely event that one of our products does not perform as expected, you're protected by our Product Promise.
For full details, please see our Terms & Conditions

Please note: All products are 'FOR RESEARCH USE ONLY. NOT FOR USE IN DIAGNOSTIC OR THERAPEUTIC PROCEDURES'.

For licensing inquiries, please contact partnerships@abcam.com