Metabolism Antibodies 1

Anti-COQ6 Antibody (CAB14335)

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SKU:
CAB14335
Product Type:
Antibody
Reactivity:
Human
Reactivity:
Mouse
Host Species:
Rabbit
Isotype:
IgG
Antibody Type:
Polyclonal Antibody
Research Area:
Metabolism

Description

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Antibody Name:Anti-COQ6 Antibody
Antibody SKU:CAB14335
Antibody Size:20uL, 50uL, 100uL
Application:WB
Reactivity:Human, Mouse
Host Species:Rabbit
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 169-468 of human COQ6 (NP_872282.1).
Application:WB
Recommended Dilution:WB 1:500 - 1:2000
Reactivity:Human, Mouse
Positive Samples:HT-29, A-549, Mouse stomach, Mouse brain, Mouse liver, Mouse kidney
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 169-468 of human COQ6 (NP_872282.1).
Purification Method:Affinity purification
Storage Buffer:Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:IgG
Sequence:SKAI RYTW PCPF PMAD SSPW VHIT LGDG STFQ TKLL IGAD GHNS GVRQ AVGI QNVS WNYD QSAV VATL HLSE ATEN NVAW QRFL PSGP IALL PLSD TLSS LVWS TSHE HAAE LVSM DEEK FVDA VNSA FWSD ADHT DFID TAGA MLQY AVSL LKPT KVSA RQLP PSVA RVDA KSRV LFPL GLGH AAEY VRPR VALI GDAA HRVH PLAG QGVN MGFG DISS LAHH LSTA AFNG KDLG SVSH LTGY ETER QRHN TALL AATD LLKR LYST SASP LVLL RTWG LQAT NAVS PLKE QIMA FASK
Gene ID:51004
Uniprot:Q9Y2Z9
Cellular Location:Cell projection, Golgi apparatus, Matrix side, Mitochondrion inner membrane, Peripheral membrane protein
Calculated MW:42kDa/48kDa/50kDa
Observed MW:51kDa
Synonyms:COQ6, CGI-10, CGI10, COQ10D6, coenzyme Q6
Background:The protein encoded by this gene belongs to the ubiH/COQ6 family. It is an evolutionarily conserved monooxygenase required for the biosynthesis of coenzyme Q10 (or ubiquinone), which is an essential component of the mitochondrial electron transport chain, and one of the most potent lipophilic antioxidants implicated in the protection of cell damage by reactive oxygen species. Knockdown of this gene in mouse and zebrafish results in decreased growth due to increased apoptosis. Mutations in this gene are associated with autosomal recessive coenzyme Q10 deficiency-6 (COQ10D6), which manifests as nephrotic syndrome with sensorineural deafness. Alternatively spliced transcript variants encoding different isoforms have been described for this gene.
UniProt Protein Function:COQ6: Defects in COQ6 are the cause of coenzyme Q10 deficiency, primary, type 6 (COQ10D6). An autosomal recessive disorder characterized by onset in infancy of severe progressive nephrotic syndrome resulting in end-stage renal failure and sensorineural deafness. Renal biopsy usually shows focal segmental glomerulosclerosis. Belongs to the UbiH/COQ6 family. 3 isoforms of the human protein are produced by alternative splicing.
UniProt Protein Details:

Protein type:Cofactor and Vitamin Metabolism - ubiquinone and other terpenoid-quinone biosynthesis; EC 1.14.13.-; Mitochondrial; Oxidoreductase

Chromosomal Location of Human Ortholog: 14q24.3

Cellular Component: mitochondrion

Disease: Coenzyme Q10 Deficiency, Primary, 6

NCBI Summary:The protein encoded by this gene belongs to the ubiH/COQ6 family. It is an evolutionarily conserved monooxygenase required for the biosynthesis of coenzyme Q10 (or ubiquinone), which is an essential component of the mitochondrial electron transport chain, and one of the most potent lipophilic antioxidants implicated in the protection of cell damage by reactive oxygen species. Knockdown of this gene in mouse and zebrafish results in decreased growth due to increased apoptosis. Mutations in this gene are associated with autosomal recessive coenzyme Q10 deficiency-6 (COQ10D6), which manifests as nephrotic syndrome with sensorineural deafness. Alternatively spliced transcript variants encoding different isoforms have been described for this gene. [provided by RefSeq, Jun 2012]
UniProt Code:Q9Y2Z9
NCBI GenInfo Identifier:26006952
NCBI Gene ID:51004
NCBI Accession:Q9Y2Z9.2
UniProt Secondary Accession:Q9Y2Z9,Q53GG6, Q86U30, Q96CA1, Q96CK2, B7Z3K8,
UniProt Related Accession:Q9Y2Z9
Molecular Weight:51kDa
NCBI Full Name:Ubiquinone biosynthesis monooxygenase COQ6, mitochondrial
NCBI Synonym Full Names:coenzyme Q6, monooxygenase
NCBI Official Symbol:COQ6  
NCBI Official Synonym Symbols:CGI10; CGI-10; COQ10D6  
NCBI Protein Information:ubiquinone biosynthesis monooxygenase COQ6, mitochondrial
UniProt Protein Name:Ubiquinone biosynthesis monooxygenase COQ6, mitochondrial
UniProt Synonym Protein Names:Coenzyme Q10 monooxygenase 6
UniProt Gene Name:COQ6  
Anti-COQ6 Antibody (CAB14335)Western blot analysis of extracts of various cell lines, using COQ6 antibody (CAB14335) at 1:3000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 90s.
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