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Recombinant Human Methylmalonic aciduria type A protein, mitochondrial (MMAA)

ACP15150

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Specifications

Cat.No	ACP15150	Target Name	MMAA
Target Synonyms	MMAAMethylmalonic aciduria type A protein; mitochondrial; EC 3.6.-.-	Form	Lyophilized powder
Expression System	Custom Production. Please inquire and provide the desire expression system.	Expression Range	66-418
Protein Length	Full Length of Mature Protein	Purity	>85% (SDS-PAGE)
Storage Buffer	5%-50% glycerol. Lyophilized powder form: the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, Liquid form: default storage buffer is Tris/PBS-based buffer, pH 8.0.

Immunogen Information

Target Species	Human	Uniprot ID	Q8IVH4

Background Information

Uniprot Id

Q8IVH4
Target Species

Human
Target Name

MMAA
Target Full Name

Methylmalonic aciduria type A protein, mitochondrial
Target Function

GTPase, binds and hydrolyzes GTP. Involved in intracellular vitamin B12 metabolism, mediates the transport of cobalamin (Cbl) into mitochondria for the final steps of adenosylcobalamin (AdoCbl) synthesis. Functions as a G-protein chaperone that assists AdoCbl cofactor delivery from MMAB to the methylmalonyl-CoA mutase (MMUT). Plays a dual role as both a protectase and a reactivase for MMUT. Protects MMUT from progressive inactivation by oxidation by decreasing the rate of the formation of the oxidized inactive cofactor hydroxocobalamin (OH2Cbl). Additionally acts a reactivase by promoting the replacement of OH2Cbl by the active cofactor AdoCbl, restoring the activity of MMUT in the presence and hydrolysis of GTP
Target Involvement

Methylmalonic aciduria type cblA (MMAA)
Target Subcellular Location

Mitochondrion. Cytoplasm.
Target Protein Families

SIMIBI class G3E GTPase family, ArgK/MeaB subfamily
Target Tissue Specificity

Widely expressed. Highest expression is observed in liver and skeletal muscle.
Target Synonyms

MMAAMethylmalonic aciduria type A protein; mitochondrial; EC 3.6.-.-

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Recombinant Human Methylmalonic aciduria type A protein, mitochondrial (MMAA)

Specifications

Immunogen Information

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