Catalog No.
YHN36001
Expression system
E. coli
Species
Homo sapiens (Human)
Protein length
Ser45-Ala265
Predicted molecular weight
27.18 kDa
Nature
Recombinant
Endotoxin level
Please contact with the lab for this information.
Purity
>90% as determined by SDS-PAGE.
Accession
Q9Y3A0
Applications
ELISA, Immunogen, SDS-PAGE, WB, Bioactivity testing in progress
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
COQ4, Ubiquinone biosynthesis protein COQ4 homolog, mitochondrial, Coenzyme Q biosynthesis protein 4 homolog
Two cases of neonatal hyperglycemia caused by a homozygous COQ9 stop-gain variant., PMID:40062559
Clinical features and genotype in COQ4 associated hereditary spastic paraplegia: a case report and a literature reanalysis., PMID:39776381
Alterations in coenzyme Q10 status in a cybrid line harboring the 3243A>G mutation of mitochondrial DNA is associated with abnormal mitochondrial bioenergetics and dysregulated mitochondrial biogenesis., PMID:38960080
Comprehensive analysis of lactate-related gene profiles and immune characteristics in lupus nephritis., PMID:38455045
In vitro construction of the COQ metabolon unveils the molecular determinants of coenzyme Q biosynthesis., PMID:38425362
COQ4 is required for the oxidative decarboxylation of the C1 carbon of coenzyme Q in eukaryotic cells., PMID:38295803
Biallelic COQ4 Variants in Hereditary Spastic Paraplegia: Clinical and Molecular Characterization., PMID:38014483
COQ4 is required for the oxidative decarboxylation of the C1 carbon of Coenzyme Q in eukaryotic cells., PMID:38014142
Biallelic variants in the COQ4 gene caused hereditary spastic paraplegia predominant phenotype., PMID:38013626
Epilepsy and Coenzyme Q10 deficiency with COQ4 variants., PMID:37948995
Ethnically unique disease burden and limitations of current expanded carrier screening panels., PMID:37681470
Bi-Allelic COQ4 Variants Cause Adult-Onset Ataxia-Spasticity Spectrum Disease., PMID:36047608
Levels of Coenzyme Q10 and Several COQ Proteins in Human Astrocytoma Tissues Are Inversely Correlated with Malignancy., PMID:35204836
Human COQ4 deficiency: delineating the clinical, metabolic and neuroimaging phenotypes., PMID:34656997
Diagnostic yield of whole-exome sequencing in non-syndromic intellectual disability., PMID:33739554
New pathogenic variants in COQ4 cause ataxia and neurodevelopmental disorder without detectable CoQ10 deficiency in muscle or skin fibroblasts., PMID:33704555
Clinical spectrum in multiple families with primary COQ10 deficiency., PMID:33215859
[Primary coenzyme Q10 deficiency-7: a case report and literature review]., PMID:33120466
Delineation of molecular findings by whole-exome sequencing for suspected cases of paediatric-onset mitochondrial diseases in the Southern Chinese population., PMID:32907636
Characterization of human mitochondrial PDSS and COQ proteins and their roles in maintaining coenzyme Q10 levels and each other's stability., PMID:32194061
COQ4 Mutation Leads to Childhood-Onset Ataxia Improved by CoQ10 Administration., PMID:30847826
Clinical phenotype, in silico and biomedical analyses, and intervention for an East Asian population-specific c.370G>A (p.G124S) COQ4 mutation in a Chinese family with CoQ10 deficiency-associated Leigh syndrome., PMID:30659264
PARL deficiency in mouse causes Complex III defects, coenzyme Q depletion, and Leigh-like syndrome., PMID:30578322
Skeletal muscle mitochondrial bioenergetics and associations with myostatin genotypes in the Thoroughbred horse., PMID:29190290
Detection of 6-demethoxyubiquinone in CoQ10 deficiency disorders: Insights into enzyme interactions and identification of potential therapeutics., PMID:28552678
Genetic Rescue of Mitochondrial and Skeletal Muscle Impairment in an Induced Pluripotent Stem Cells Model of Coenzyme Q10 Deficiency., PMID:28472853
Generation, genome edition and characterization of iPSC lines from a patient with coenzyme Q10 deficiency harboring a heterozygous mutation in COQ4 gene., PMID:28465093
Amitriptyline down-regulates coenzyme Q10 biosynthesis in lung cancer cells., PMID:28099869
Diagnostic exome sequencing provides a molecular diagnosis for a significant proportion of patients with epilepsy., PMID:26795593
Mutations in COQ4, an essential component of coenzyme Q biosynthesis, cause lethal neonatal mitochondrial encephalomyopathy., PMID:26185144
Biosynthesis of coenzyme Q in eukaryotes., PMID:26183239
Yeast Coq9 controls deamination of coenzyme Q intermediates that derive from para-aminobenzoic acid., PMID:26008578
COQ4 mutations cause a broad spectrum of mitochondrial disorders associated with CoQ10 deficiency., PMID:25658047
The regulation of coenzyme q biosynthesis in eukaryotic cells: all that yeast can tell us., PMID:25126044
Coenzyme Q supplementation or over-expression of the yeast Coq8 putative kinase stabilizes multi-subunit Coq polypeptide complexes in yeast coq null mutants., PMID:24406904
nde1 deletion improves mitochondrial DNA maintenance in Saccharomyces cerevisiae coenzyme Q mutants., PMID:23116202
Ter-dependent stress response systems: novel pathways related to metal sensing, production of a nucleoside-like metabolite, and DNA-processing., PMID:23044854
Overexpression of the Coq8 kinase in Saccharomyces cerevisiae coq null mutants allows for accumulation of diagnostic intermediates of the coenzyme Q6 biosynthetic pathway., PMID:22593570
Haploinsufficiency of COQ4 causes coenzyme Q10 deficiency., PMID:22368301
Genetic variability of transcript abundance in pig peri-mortem skeletal muscle: eQTL localized genes involved in stress response, cell death, muscle disorders and metabolism., PMID:22053791
A Drosophila model for primary coenzyme Q deficiency and dietary rescue in the developing nervous system., PMID:20889762
The yeast Coq4 polypeptide organizes a mitochondrial protein complex essential for coenzyme Q biosynthesis., PMID:19022396
Hydroxylation of demethoxy-Q6 constitutes a control point in yeast coenzyme Q6 biosynthesis., PMID:19002377
Genetic polymorphisms and preliminary association analysis with production traits of the porcine SLC27A4 gene., PMID:18696256
Functional characterization of human COQ4, a gene required for Coenzyme Q10 biosynthesis., PMID:18474229
Saccharomyces cerevisiae Coq9 polypeptide is a subunit of the mitochondrial coenzyme Q biosynthetic complex., PMID:17391640
Complementation of Saccharomyces cerevisiae coq7 mutants by mitochondrial targeting of the Escherichia coli UbiF polypeptide: two functions of yeast Coq7 polypeptide in coenzyme Q biosynthesis., PMID:16624818
Coq3 and Coq4 define a polypeptide complex in yeast mitochondria for the biosynthesis of coenzyme Q., PMID:15792955
For research use only. Not for human or drug use.
