Metabolism Antibodies 1
Anti-COQ9 Antibody (CAB15480)
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- SKU:
- CAB15480
- Product Type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Metabolism
Description
| Antibody Name: | Anti-COQ9 Antibody |
| Antibody SKU: | CAB15480 |
| Antibody Size: | 20uL, 50uL, 100uL |
| Application: | WB |
| Reactivity: | Human |
| Host Species: | Rabbit |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-318 of human COQ9 (NP_064708.1). |
| Application: | WB |
| Recommended Dilution: | WB 1:200 - 1:2000 |
| Reactivity: | Human |
| Positive Samples: | A-549, A431, HeLa, LO2 |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-318 of human COQ9 (NP_064708.1). |
| 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: | MAAA AVSG ALGR AGWR LLQL RCLP VARC RQAL VPRA FHAS AVGL RSSD EQKQ QPPN SFSQ QHSE TQGA EKPD PESS HSPP RYTD QGGE EEED YESE EQLQ HRIL TAAL EFVP AHGW TAEA IAEG AQSL GLSS AAAS MFGK DGSE LILH FVTQ CNTR LTRV LEEE QKLV QLGQ AEKR KTDQ FLRD AVET RLRM LIPY IEHW PRAL SILM LPHN IPSS LSLL TSMV DDMW HYAG DQST DFNW YTRR AMLA AIYN TTEL VMMQ DSSP DFED TWRF LENR VNDA MNMG HTAK QVKS TGEA LVQG LMGA AVTL KNLT GLNQ RR |
| Gene ID: | 57017 |
| Uniprot: | O75208 |
| Cellular Location: | Mitochondrion |
| Calculated MW: | 14kDa/35kDa |
| Observed MW: | 36kDa |
| Synonyms: | COQ9, C16orf49, COQ10D5 |
| Background: | This locus represents a mitochondrial ubiquinone biosynthesis gene. The encoded protein is likely necessary for biosynthesis of coenzyme Q10, as mutations at this locus have been associated with autosomal-recessive neonatal-onset primary coenzyme Q10 deficiency. |
| UniProt Protein Function: | COQ9: Involved in the biosynthesis of coenzyme Q. Defects in COQ9 are the cause of coenzyme Q10 deficiency, primary, type 5 (COQ10D5). A form of coenzyme Q10 deficiency, an autosomal recessive disorder with variable manifestations consistent with 5 major phenotypes. The phenotypes include an encephalomyopathic form with seizures and ataxia; a multisystem infantile form with encephalopathy, cardiomyopathy and renal failure; a predominantly cerebellar form with ataxia and cerebellar atrophy; Leigh syndrome with growth retardation; and an isolated myopathic form. Belongs to the COQ9 family. 2 isoforms of the human protein are produced by alternative splicing. |
| UniProt Protein Details: | Protein type:Mitochondrial Chromosomal Location of Human Ortholog: 16q21 Cellular Component: mitochondrial inner membrane; mitochondrion Molecular Function:lipid binding; protein binding; protein homodimerization activity Biological Process: ubiquinone biosynthetic process Disease: Coenzyme Q10 Deficiency, Primary, 5 |
| NCBI Summary: | This locus represents a mitochondrial ubiquinone biosynthesis gene. The encoded protein is likely necessary for biosynthesis of coenzyme Q10, as mutations at this locus have been associated with autosomal-recessive neonatal-onset primary coenzyme Q10 deficiency.[provided by RefSeq, Sep 2010] |
| UniProt Code: | O75208 |
| NCBI GenInfo Identifier: | 74735505 |
| NCBI Gene ID: | 57017 |
| NCBI Accession: | O75208.1 |
| UniProt Secondary Accession: | O75208,Q7L5V7, Q7Z5T6, Q8NBL4, Q9NTJ2, Q9P056, A8K3L2 |
| UniProt Related Accession: | O75208 |
| Molecular Weight: | 14,505 Da |
| NCBI Full Name: | Ubiquinone biosynthesis protein COQ9, mitochondrial |
| NCBI Synonym Full Names: | coenzyme Q9 |
| NCBI Official Symbol: | COQ9 |
| NCBI Official Synonym Symbols: | COQ10D5; C16orf49 |
| NCBI Protein Information: | ubiquinone biosynthesis protein COQ9, mitochondrial |
| UniProt Protein Name: | Ubiquinone biosynthesis protein COQ9, mitochondrial |
| Protein Family: | Ubiquinone biosynthesis protein |
| UniProt Gene Name: | COQ9 |
| UniProt Entry Name: | COQ9_HUMAN |
 | Western blot analysis of extracts of various cell lines, using COQ9 antibody (CAB15480) at 1:1000 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: 10s. |
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