ACAD9 Antibody, FITC conjugated

Code: CSB-PA887999EC01HU

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50μg100μg

US$166

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Species Reactivity: Human

Raised in: Rabbit

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Target Background

Product Details

Uniprot NO.

Q9H845

Target Names

ACAD9

Alternative Names

ACAD9Complex I assembly factor ACAD9 antibody; mitochondrial antibody; Acyl-CoA dehydrogenase family member 9 antibody; ACAD-9 antibody; EC 1.3.8.- antibody

Raised in

Rabbit

Species Reactivity

Human

Immunogen

Recombinant Human Acyl-CoA dehydrogenase family member 9, mitochondrial protein (1-270AA)

Immunogen Species

Homo sapiens (Human)

Conjugate

FITC

Isotype

IgG

Purification Method

>95%, Protein G purified

Concentration

It differs from different batches. Please contact us to confirm it.

Buffer

Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4

Storage

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.

Lead Time

Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.

Usage

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

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Target Background

Function(From Uniprot)

As part of the MCIA complex, primarily participates in the assembly of the mitochondrial complex I and therefore plays a role in oxidative phosphorylation. This moonlighting protein has also a dehydrogenase activity toward a broad range of substrates with greater specificity for long-chain unsaturated acyl-CoAs. However, in vivo, it does not seem to play a primary role in fatty acid oxidation. In addition, the function in complex I assembly is independent of the dehydrogenase activity of the protein.

Gene References into Functions

Mutations in the ND6, NDUFV1 or ACAD9 genes are responsible for the mitochondrial complex I deficiency. PMID:29348607
Study identified new mutations in ACAD9 responsible for a wide spectrum of heart diseases in the presence of elevated serum lactate levels. PMID:27233227
ACAD9 mutation is the most frequent cause of cardiac hypertrophy and isolated complex I deficiency. PMID:26669660
Case Report: neonatal multiorgan failure due to ACAD9 mutation and complex I deficiency with mitochondrial hyperplasia in liver, cardiac myocytes, skeletal muscle, and renal tubules. PMID:26826406
In cells where it is strongly expressed, ACAD9 plays a physiological role in fatty acid oxidation. PMID:25721401
Our results underscore the importance of the ACAD9 protein in complex I assembly and suggest that the enzymatic activity is a rudiment of the duplication event. PMID:24158852
Our data support a new function for ACAD9 in complex I function, making this gene an important new candidate for patients with complex I deficiency, which could be improved by riboflavin treatment. PMID:20929961
ACAD9 screening of 120 additional complex I-defective index cases led us to identify two additional unrelated cases and a total of five pathogenic ACAD9 alleles. PMID:21057504
Data show that two closely related metabolic enzymes, ACAD9 and VLCAD, diverged at the root of the vertebrate lineage to function in two separate mitochondrial metabolic pathways and have clinical implications for the diagnosis of complex I deficiency. PMID:20816094
Very high activity of CPT2 and VCLAD, involved in the metabolism of long-chain fatty acids. Fatty acid oxidation may play role in energy generation in placenta, and deficiency in may result in placental dysfunction and gestational complications. PMID:12971426
ACAD9 may play a role in the turnover of lipid membrane unsaturated fatty acids that are essential for membrane integrity and structure PMID:16020546
acyl-CoA dehydrogenase 9 (ACAD 9)was identified as the long-chain ACAD in human embryonic and fetal brain and central nervous tissue, using in situ hybridization as well as enzymatic studies PMID:16750164
We now report three cases of ACAD9 deficiency. PMID:17564966
Accumulation of 3-hydroxylated intermediates of long-chain fatty acids may contribute to the pathogenesis of retinopathy in MTP deficiencies. PMID:18385088
Validated occurrence of an unusual TG 3' splice site in intron 10. PMID:17672918

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Subcellular Location

Mitochondrion inner membrane; Peripheral membrane protein; Matrix side.

Protein Families

Acyl-CoA dehydrogenase family

Tissue Specificity

Ubiquitously expressed in most normal human tissues and cancer cell lines with high level of expression in heart, skeletal muscles, brain, kidney and liver. In the cerebellum uniquely expressed in the granular layer (at protein level).

Database Links

HGNC: 21497

UNIGENE: Hs.567482

KEGG: hsa:28976

STRING: 9606.ENSP00000312618

OMIM: 611103

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