Recombinant Human Ubiquinone biosynthesis protein COQ4 homolog, mitochondrial (COQ4)

中文名稱：

Recombinant Human Ubiquinone biosynthesis protein COQ4 homolog, mitochondrial(COQ4)
貨號：

CSB-YP896885HU
說明書：

MSDS Datasheet
規格：
來源：

Yeast
其他：

中文名稱：

Recombinant Human Ubiquinone biosynthesis protein COQ4 homolog, mitochondrial(COQ4)
貨號：

CSB-EP896885HU
說明書：

MSDS Datasheet
規格：
來源：

E.coli
其他：

中文名稱：

Recombinant Human Ubiquinone biosynthesis protein COQ4 homolog, mitochondrial(COQ4)
貨號：

CSB-EP896885HU-B
說明書：

MSDS Datasheet
規格：
來源：

E.coli
共軛：

Avi-tag Biotinylated
E. coli biotin ligase (BirA) is highly specific in covalently attaching biotin to the 15 amino acid AviTag peptide. This recombinant protein was biotinylated in vivo by AviTag-BirA technology, which method is BriA catalyzes amide linkage between the biotin and the specific lysine of the AviTag.
其他：

中文名稱：

Recombinant Human Ubiquinone biosynthesis protein COQ4 homolog, mitochondrial(COQ4)
貨號：

CSB-BP896885HU
說明書：

MSDS Datasheet
規格：
來源：

Baculovirus
其他：

中文名稱：

Recombinant Human Ubiquinone biosynthesis protein COQ4 homolog, mitochondrial(COQ4)
貨號：

CSB-MP896885HU
說明書：

MSDS Datasheet
規格：
來源：

Mammalian cell
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

純度：

>85% (SDS-PAGE)
基因名：

COQ4
Uniprot No.：

Q9Y3A0
別名：

CGI 92; Coenzyme Q biosynthesis protein 4 homolog; coq4; COQ4_HUMAN; mitochondrial; Ubiquinone biosynthesis protein COQ4 homolog
種屬：

Homo sapiens (Human)
蛋白長度：

full length protein
表達區域：

31-265
氨基酸序列

SDGAGPLYSH HLPTSPLQKG LLAAGSAAMA LYNPYRHDMV AVLGETTGHR TLKVLRDQMR RDPEGAQILQ ERPRISTSTL DLGKLQSLPE GSLGREYLRF LDVNRVSPDT RAPTRFVDDE ELAYVIQRYR EVHDMLHTLL GMPTNILGEI VVKWFEAVQT GLPMCILGAF FGPIRLGAQS LQVLVSELIP WAVQNGRRAP CVLNLYYERR WEQSLRALRE ELGITAPPMH VQGLA
蛋白標簽：

Tag?type?will?be?determined?during?the?manufacturing?process.

The tag type will be determined during production process. If you have specified tag type, please tell us and we will develop the specified tag preferentially.
產品提供形式：

Lyophilized powder
Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
復溶：

We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20℃/-80℃. Our default final concentration of glycerol is 50%. Customers could use it as reference.
儲存條件：

Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
保質期：

The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
貨期：

Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.
注意事項：

Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
Datasheet ：

Please contact us to get it.

產品評價

靶點詳情

功能：

Component of the coenzyme Q biosynthetic pathway. May play a role in organizing a multi-subunit COQ enzyme complex required for coenzyme Q biosynthesis. Required for steady-state levels of other COQ polypeptides.
基因功能參考文獻：
1. The COQ4 mutation was CRISPR/Cas9 edited resulting in isogenic, diploid and off-target free COQ4-corrected iPSCs. PMID: 28465093
2. Three genes in our epilepsy cohort (COQ4, DNM1, and PURA), accounting for 14% (3/21) of all novel genetic etiologies identified in patients with epilepsy, were subsequently confirmed in independent publications. PMID: 26795593
3. five recessive missense mutations in COQ4 segregating with lethal neonatal mitochondrial encephalomyopathy in four families of Ashkenzi Jews PMID: 26185144
4. COQ4 mutations cause a broad spectrum of mitochondrial disorders associated with CoQ10 deficiency. PMID: 25658047
5. Haploinsufficiency in COQ4 resulted in reduced COQ4 expression, CoQ10 content and biosynthetic rate, and activities of respiratory chain complex II+III. PMID: 22368301
6. Human ortholog of Saccharomyces cerevisiae COQ4 was cloned and characterized. PMID: 18474229
7. homologous to Coq4p in S.cerevisiae PMID: 11469793
顯示更多

收起更多
相關疾?。?/div>
Coenzyme Q10 deficiency, primary, 7 (COQ10D7)
亞細胞定位：

[Isoform 1]: Mitochondrion inner membrane; Peripheral membrane protein; Matrix side.
蛋白家族：

COQ4 family
組織特異性：

Expressed ubiquitously, but at high levels in liver, lung and pancreas.
數據庫鏈接：

HGNC: 19693

OMIM: 612898

KEGG: hsa:51117

STRING: 9606.ENSP00000300452

UniGene: Hs.98541