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AB163430

Recombinant Human MCCC1 protein

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Recombinant Human MCCC1 protein is a Human Full Length protein, in the 1 to 725 aa range, expressed in Wheat germ, suitable for ELISA, WB.

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

1 Images
SDS-PAGE - Recombinant Human MCCC1 protein (AB163430)
  • SDS-PAGE

Unknown

SDS-PAGE - Recombinant Human MCCC1 protein (AB163430)

ab163430 on a 12.5% SDS-PAGE stained with Coomassie Blue.

Key facts

Expression system

Wheat germ

Tags

GST tag N-Terminus

Applications

WB, ELISA

applications

Biologically active

No

Accession

Q96RQ3

Animal free

No

Carrier free

No

Species

Human

Storage buffer

pH: 8 Constituents: 0.79% Tris HCl, 0.31% Glutathione

storage-buffer

Reactivity data

{ "title": "Reactivity Data", "filters": { "stats": ["", "Reactivity", "Dilution Info", "Notes"] }, "values": { "ELISA": { "reactivity":"TESTED_AND_REACTS", "dilution-info":"", "notes":"<p></p>" }, "WB": { "reactivity":"TESTED_AND_REACTS", "dilution-info":"", "notes":"<p></p>" } } }

Sequence info

[{"sequence":"MAAASAVSVLLVAAERNRWHRLPSLLLPPRTWVWRQRTMKYTTATGRNITKVLIANRGEIACRVMRTAKKLGVQTVAVYSEADRNSMHVDMADEAYSIGPAPSQQSYLSMEKIIQVAKTSAAQAIHPGCGFLSENMEFAELCKQEGIIFIGPPPSAIRDMGIKSTSKSIMAAAGVPVVEGYHGEDQSDQCLKEHARRIGYPVMIKAVRGGGGKGMRIVRSEQEFQEQLESARREAKKSFNDDAMLIEKFVDTPRHVEVQVFGDHHGNAVYLFERDCSVQRRHQKIIEEAPAPGIKSEVRKKLGEAAVRAAKAVNYVGAGTVEFIMDSKHNFCFMEMNTRLQVEHPVTEMITGTDLVEWQLRIAAGEKIPLSQEEITLQGHAFEARIYAEDPSNNFMPVAGPLVHLSTPRADPSTRIETGVRQGDEVSVHYDPMIAKLVVWAADRQAALTKLRYSLRQYNIVGLPTNIDFLLNLSGHPEFEAGNVHTDFIPQHHKQLLLSRKAAAKESLCQAALGLILKEKAMTDTFTLQAHDQFSPFSSSSGRRLNISYTRNMTLKDGKNNVAIAVTYNHDGSYSMQIEDKTFQVLGNLYSEGDCTYLKCSVNGVASKAKLIILENTIYLFSKEGSIEIDIPVPKYLSSVSSQETQGGPLAPMTGTIEKVFVKAGDKVKAGDSLMVMIAMKMEHTIKSPKDGTVKKVFYREGAQANRHTPLVEFEEEESDKRESE","proteinLength":"Full Length","predictedMolecularWeight":null,"actualMolecularWeight":null,"aminoAcidEnd":725,"aminoAcidStart":1,"nature":"Recombinant","expressionSystem":"Wheat germ","accessionNumber":"Q96RQ3","tags":[{"tag":"GST","terminus":"N-Terminus"}]}]

Properties and storage information

Shipped at conditions
Dry Ice
Appropriate short-term storage conditions
-80°C
Appropriate long-term storage conditions
-80°C
Aliquoting information
Upon delivery aliquot
Storage information
Avoid freeze / thaw cycle
False

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.

Specifications

Form

Liquid

General info

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.

Post-translational modifications

Acetylated.

Subcellular localisation

Mitochondrion matrix

Product protocols

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

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