|Positive WB detected in||mouse liver tissue, Transfected HEK-293 cells|
|Positive IP detected in||mouse liver tissue|
|Positive IHC detected in||human liver tissue, human pancreas tissue|
Note: suggested antigen retrieval with TE buffer pH 9.0; (*) Alternatively, antigen retrieval may be performed with citrate buffer pH 6.0
|Western Blot (WB)||WB : 1:500-1:2000|
|Immunoprecipitation (IP)||IP : 0.5-4.0 ug for IP and 1:500-1:1000 for WB|
|Immunohistochemistry (IHC)||IHC : 1:50-1:500|
|Sample-dependent, check data in validation data gallery|
16654-1-AP targets COQ4 in WB, IP, IHC, ELISA applications and shows reactivity with human, mouse, rat samples.
|Tested Reactivity||human, mouse, rat|
|Cited Reactivity||human, mouse|
|Host / Isotype||Rabbit / IgG|
|Immunogen||COQ4 fusion protein Ag10089|
|Full Name||coenzyme Q4 homolog (S. cerevisiae)|
|Calculated molecular weight||265 aa, 30 kDa|
|Observed molecular weight||32 kDa|
|GenBank accession number||BC011895|
|Gene ID (NCBI)||51117|
|Purification Method||Antigen affinity purification|
|Storage Buffer||PBS with 0.02% sodium azide and 50% glycerol pH 7.3.|
|Storage Conditions||Store at -20°C. Stable for one year after shipment. Aliquoting is unnecessary for -20oC storage. 20ul sizes contain 0.1% BSA.|
EMBO Mol Med
β-RA reduces DMQ/CoQ ratio and rescues the encephalopathic phenotype in Coq9 R239X mice.
The Q-junction and the inflammatory response are critical pathological and therapeutic factors in CoQ deficiency.
Ann Clin Transl Neurol
4-Hydroxybenzoic acid restores CoQ10 biosynthesis in human COQ2 deficiency.
J Med Genet
Human COQ4 deficiency: delineating the clinical, metabolic and neuroimaging phenotypes.
Biochim Biophys Acta Bioenerg
Characterization of human mitochondrial PDSS and COQ proteins and their roles in maintaining coenzyme Q10 levels and each other's stability.
Mol Genet Metab
Detection of 6-demethoxyubiquinone in CoQ10 deficiency disorders: Insights into enzyme interactions and identification of potential therapeutics.