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AB174555

Recombinant Human ACAD8 protein

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Recombinant Human ACAD8 protein is a Human Full Length protein, in the 23 to 415 aa range, expressed in Escherichia coli, with >95%, suitable for SDS-PAGE, Mass Spec.

View Alternative Names

ARC42, IBD, ACAD8, IBDH, Activator-recruited cofactor 42 kDa component, Acyl-CoA dehydrogenase family member 8, ACAD-8

1 Images
SDS-PAGE - Recombinant Human ACAD8 protein (AB174555)
  • SDS-PAGE

Supplier Data

SDS-PAGE - Recombinant Human ACAD8 protein (AB174555)

15% SDS-PAGE analysis of ab174555 (3μg).

Key facts

Purity

>95% SDS-PAGE

Expression system

Escherichia coli

Tags

His tag N-Terminus

Applications

Mass Spec, SDS-PAGE

applications

Biologically active

No

Accession

Q9UKU7

Animal free

No

Carrier free

No

Species

Human

Storage buffer

pH: 8 Constituents: 30% Glycerol (glycerin, glycerine), 0.88% Sodium chloride, 0.32% Tris HCl, 0.02% (R*,R*)-1,4-Dimercaptobutan-2,3-diol

storage-buffer

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Reactivity data

{ "title": "Reactivity Data", "filters": { "stats": ["", "Reactivity", "Dilution Info", "Notes"] }, "values": { "SDS-PAGE": { "reactivity":"TESTED_AND_REACTS", "dilution-info":"", "notes":"<p></p>" }, "Mass Spec": { "reactivity":"TESTED_AND_REACTS", "dilution-info":"", "notes":"<p></p>" } } }
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Sequence info

[{"sequence":"MGSSHHHHHHSSGLVPRGSHMGSLVQTGHRSLTSCIDPSMGLNEEQKEFQKVAFDFAAREMAPNMAEWDQKELFPVDVMRKAAQLGFGGVYIQTDVGGSGLSRLDTSVIFEALATGCTSTTAYISIHNMCAWMIDSFGNEEQRHKFCPPLCTMEKFASYCLTEPGSGSDAASLLTSAKKQGDHYILNGSKAFISGAGESDIYVVMCRTGGPGPKGISCIVVEKGTPGLSFGKKEKKVGWNSQPTRAVIFEDCAVPVANRIGSEGQGFLIAVRGLNGGRINIASCSLGAAHASVILTRDHLNVRKQFGEPLASNQYLQFTLADMATRLVAARLMVRNAAVALQEERKDAVALCSMAKLFATDECFAICNQALQMHGGYGYLKDYAVQQYVRDSRVHQILEGSNEVMRILISRSLLQE","proteinLength":"Full Length","predictedMolecularWeight":"45.1 kDa","actualMolecularWeight":null,"aminoAcidEnd":415,"aminoAcidStart":23,"nature":"Recombinant","expressionSystem":"Escherichia coli","accessionNumber":"Q9UKU7","tags":[{"tag":"His","terminus":"N-Terminus"}]}]
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Properties and storage information

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
False
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Supplementary information

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

ACAD8 also known as Acyl-CoA dehydrogenase family member 8 is an enzyme with a mass of approximately 46 kDa. It functions by catalyzing the initial step in the mitochondrial fatty acid β-oxidation specifically handling the short-branched chain acyl-CoA substrates. ACAD8 predominantly expresses in tissues with high mitochondrial content such as the liver heart and muscle. The enzyme plays a critical role in energy production by breaking down certain fatty acids making it essential for the cellular energy balance.
Biological function summary

The enzyme facilitates the metabolism of branched-chain amino acids converting them into simpler compounds that the body can use for energy and growth. ACAD8 operates as part of a larger multienzyme complex known as the mitochondrial trifunctional protein. The dehydrogenase transfers electrons derived from substrate metabolism to electron transport chain components via ETF (electron transfer flavoprotein) assisting in energy production. This process is vital for the proper functioning of cells especially in tissues demanding constant energy.

Pathways

ACAD8 participates in the leucine degradation pathway and is critical in the mitochondrial fatty acid β-oxidation process. It works closely with other enzymes in the acyl-CoA dehydrogenase family contributing to energy homeostasis. Sufficient functioning of ACAD8 is necessary for the conversion of branched-chain amino acids to acyl-CoA derivatives which are ultimately processed through the electron transport chain to produce ATP. Any disruption in these pathways can result in metabolic challenges and energy generation problems.

Mutations or deficiencies in ACAD8 can lead to disorders such as isobutyryl-CoA dehydrogenase deficiency (IBD deficiency) which is a rare metabolic condition affecting branched-chain amino acid metabolism. Patients with malfunctioning ACAD8 often show elevated levels of isobutyryl-carnitine due to the impaired breakdown process. While direct links to specific disorders might involve other proteins like the ETF alpha and beta subunits ACAD8's deficiency primarily influences the metabolic balance concerning fatty acid breakdown and energy production. Understanding this relationship is important to diagnose and manage conditions related to defective fatty acid oxidation.
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Specifications

Form

Liquid

Additional notes

ab174555 is purified using conventional chromatography techniques.

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General info

Function

Isobutyryl-CoA dehydrogenase which catalyzes the conversion of 2-methylpropanoyl-CoA to (2E)-2-methylpropenoyl-CoA in the valine catabolic pathway (PubMed : 11013134, PubMed : 12359132, PubMed : 16857760). To a lesser extent, also able to catalyze the oxidation of (2S)-2-methylbutanoyl-CoA (PubMed : 11013134, PubMed : 12359132).

Sequence similarities

Belongs to the acyl-CoA dehydrogenase family.

Subcellular localisation

Mitochondrion

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Product protocols

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Target data

Isobutyryl-CoA dehydrogenase which catalyzes the conversion of 2-methylpropanoyl-CoA to (2E)-2-methylpropenoyl-CoA in the valine catabolic pathway (PubMed : 11013134, PubMed : 12359132, PubMed : 16857760). To a lesser extent, also able to catalyze the oxidation of (2S)-2-methylbutanoyl-CoA (PubMed : 11013134, PubMed : 12359132).
See full target information ACAD8
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