Catalog No.
YHK55501
Expression system
E. coli
Species
Homo sapiens (Human)
Protein length
Thr11-Asn429
Predicted molecular weight
48.26 kDa
Nature
Recombinant
Applications
ELISA, Immunogen, SDS-PAGE, WB, Bioactivity testing in progress
Endotoxin level
Please contact with the lab for this information.
Purity
>90% as determined by SDS-PAGE.
Accession
Q9H845
Form
Lyophilized
Storage buffer
Lyophilized from a solution in PBS pH 7.4, 0.02% NLS, 1mM EDTA, 4% Trehalose, 1% Mannitol.
Reconstitution
Reconstitute in sterile water for a stock solution. A copy of datasheet will be provided with the products, please refer to it for details.
Shipping
In general, proteins are provided as lyophilized powder/frozen liquid. They are shipped out with dry ice/blue ice unless customers require otherwise.
Stability and Storage
Use a manual defrost freezer and avoid repeated freeze thaw cycles. Store at 2 to 8°C for frequent use. Store at -20 to -80°C for twelve months from the date of receipt.
Alternative Names
ACAD9, Acyl-CoA dehydrogenase family member 9, ACAD-9, Complex I assembly factor ACAD9, mitochondrial
Note
For research use only.
Impaired complex-I mitochondrial biogenesis in Parkinson disease frontal cortex., PMID:23939409
Update on clinical aspects and treatment of selected vitamin-responsive disorders II (riboflavin and CoQ 10)., PMID:22231380
Molecular diagnosis in mitochondrial complex I deficiency using exome sequencing., PMID:22499348
Complexome profiling identifies TMEM126B as a component of the mitochondrial complex I assembly complex., PMID:22982022
Mitochondrial encephalomyopathy due to a novel mutation in ACAD9., PMID:23836383
A Patient with Complex I Deficiency Caused by a Novel ACAD9 Mutation Not Responding to Riboflavin Treatment., PMID:23996478
Chronic hypoxia alters mitochondrial composition in human macrophages., PMID:24140568
ACAD9, a complex I assembly factor with a moonlighting function in fatty acid oxidation deficiencies., PMID:24158852
Very long-chain acyl-coenzyme A dehydrogenase deficiency in Chinese patients: eight case reports, including one case of prenatal diagnosis., PMID:25652019
Complex I assembly function and fatty acid oxidation enzyme activity of ACAD9 both contribute to disease severity in ACAD9 deficiency., PMID:25721401
Another "Complex" Case: Complex I Deficiency Secondary to Acyl-CoA Dehydrogenase 9 Mutation., PMID:26445304
Lethal Neonatal Progression of Fetal Cardiomegaly Associated to ACAD9 Deficiency., PMID:26475292
Mitochondrial Involvement in Vertebrate Speciation? The Case of Mito-nuclear Genetic Divergence in Chameleons., PMID:26590214
High incidence and variable clinical outcome of cardiac hypertrophy due to ACAD9 mutations in childhood., PMID:26669660
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
The origin of the supernumerary subunits and assembly factors of complex I: A treasure trove of pathway evolution., PMID:27048931
A Mutation in the Flavin Adenine Dinucleotide-Dependent Oxidoreductase FOXRED1 Results in Cell-Type-Specific Assembly Defects in Oxidative Phosphorylation Complexes I and II., PMID:27215383
Evidence of a wide spectrum of cardiac involvement due to ACAD9 mutations: Report on nine patients., PMID:27233227
Biallelic Mutations in TMEM126B Cause Severe Complex I Deficiency with a Variable Clinical Phenotype., PMID:27374774
Severe defect in mitochondrial complex I assembly with mitochondrial DNA deletions in ACAD9-deficient mild myopathy., PMID:27438479
Lifetime exercise intolerance with lactic acidosis as key manifestation of novel compound heterozygous ACAD9 mutations causing complex I deficiency., PMID:28279569
Assembly defects of multiple respiratory chain complexes in a child with cardiac hypertrophy associated with a novel ACAD9 mutation., PMID:28529009
Uncovering the embryonic development-related proteome and metabolome signatures in breast muscle and intramuscular fat of fast-and slow-growing chickens., PMID:29061108
Selection and Characterization of Palmitic Acid Responsive Patients with an OXPHOS Complex I Defect., PMID:29093663
Advances in the Understanding and Treatment of Mitochondrial Fatty Acid Oxidation Disorders., PMID:29177110
Severe riboflavin deficiency induces alterations in the hepatic proteome of starter Pekin ducks., PMID:29185933
Evaluation of mitochondrial bioenergetics, dynamics, endoplasmic reticulum-mitochondria crosstalk, and reactive oxygen species in fibroblasts from patients with complex I deficiency., PMID:29348607
Identification of a novel mitochondrial complex I assembly factor ACDH-12 in Caenorhabditis elegans., PMID:29540318
Clinical, biochemical and genetic spectrum of 70 patients with ACAD9 deficiency: is riboflavin supplementation effective?, PMID:30025539
Successful treatment of infantile-onset ACAD9-related cardiomyopathy with a combination of sodium pyruvate, beta-blocker, and coenzyme Q10., PMID:31473688
Induction of LEF1 by MYC activates the WNT pathway and maintains cell proliferation., PMID:31623618
MITRAC15/COA1 promotes mitochondrial translation in a ND2 ribosome-nascent chain complex., PMID:31721420
Dissecting the Roles of Mitochondrial Complex I Intermediate Assembly Complex Factors in the Biogenesis of Complex I., PMID:32320651
Pathway attenuation of fatty acid beta-oxidation in the skeletal muscle of a type 2 diabetic mouse model., PMID:32562559
[Human facial shape related SNP analysis in Han Chinese populations]., PMID:32694107
Assembly of The Mitochondrial Complex I Assembly Complex Suggests a Regulatory Role for Deflavination., PMID:33320993
Riboflavin in Neurological Diseases: A Narrative Review., PMID:33886098
Optic neuropathy linked to ACAD9 pathogenic variants: A potentially riboflavin-responsive disorder?, PMID:34023438
Effects of riboflavin deficiency on the lipid metabolism of duck breeders and duck embryos., PMID:34438327
Development and characterization of a mouse model for Acad9 deficiency., PMID:34556413
Molecular mechanism of interactions between ACAD9 and binding partners in mitochondrial respiratory complex I assembly., PMID:34646991
Fatty acid oxidation fuels glioblastoma radioresistance with CD47-mediated immune evasion., PMID:35314680
Molecular characteristics of the multi-functional FAO enzyme ACAD9 illustrate the importance of FADH2 /NADH ratios for mitochondrial ROS formation., PMID:35708204
Immunodeficiency with susceptibility to lymphoma with complex genotype affecting energy metabolism (FBP1, ACAD9) and vesicle trafficking (RAB27A)., PMID:37388727
Ultrasound stimulation of the vagal nerve improves acute septic encephalopathy in mice., PMID:37529234
The assembly of the Mitochondrial Complex I Assembly complex uncovers a redox pathway coordination., PMID:38086790
ACAD9 treatment with bezafibrate and nicotinamide riboside temporarily stabilizes cardiomyopathy and lactic acidosis., PMID:38797357
A Comprehensive Prognostic Model for Colon Adenocarcinoma Depending on Nuclear-Mitochondrial-Related Genes., PMID:38832431
Nuclear Genome-Encoded Mitochondrial OXPHOS Complex I Genes in Female Buffalo Show Tissue-Specific Differences., PMID:38878239
Abnormal DNA methylation of EBF1 regulates adipogenesis in chicken., PMID:40114082
For research use only. Not for human or drug use.
