Recombinant Human Putative transferase CAF17, mitochondrial (IBA57)

中文名稱：

人IBA57重組蛋白
貨號：

CSB-YP722566HU
說明書：

MSDS Datasheet
規(guī)格：
來源：

Yeast
其他：

中文名稱：

人IBA57重組蛋白
貨號：

CSB-EP722566HU
說明書：

MSDS Datasheet
規(guī)格：
來源：

E.coli
其他：

中文名稱：

人IBA57重組蛋白
貨號：

CSB-EP722566HU-B
說明書：

MSDS Datasheet
規(guī)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：

人IBA57重組蛋白
貨號：

CSB-BP722566HU
說明書：

MSDS Datasheet
規(guī)格：
來源：

Baculovirus
其他：

中文名稱：

人IBA57重組蛋白
貨號：

CSB-MP722566HU
說明書：

MSDS Datasheet
規(guī)格：
來源：

Mammalian cell
其他：

產(chǎn)品詳情
產(chǎn)品評價
相關(guān)產(chǎn)品
靶點詳情

產(chǎn)品詳情

純度：

>85% (SDS-PAGE)
基因名：

IBA57
Uniprot No.：

Q5T440
別名：

C1orf69; CAF17_HUMAN; Chromosome 1 open reading frame 69; FLJ12734; FLJ13849; IBA57; IBA57 homolog iron sulfur cluster assembly; IBA57 iron sulfur cluster assembly homolog ; IBA57 iron sulfur cluster assembly homolog (S. cerevisiae); IBA57; S. cerevisiae; homolog of; Iron sulfur cluster assembly factor for biotin synthase and aconitase like ; iron-sulfur cluster assembly factor for biotin synthase- and aconitase-like mitochondrial proteins; with a mass of 57kDa; Iron-sulfur cluster assembly factor homolog; Mitochondrial proteins with a mass of 57kDa; MMDS3; Putative transferase C1orf69 mitochondrial; Putative transferase CAF17; mitochondrial; SPG74
種屬：

Homo sapiens (Human)
蛋白長度：

Full Length of Mature Protein
表達(dá)區(qū)域：

40-356
氨基酸序列

G DPTAGAAWAC FRLDGRTLLR VRGPDAAPFL LGLLTNELPL PSPAAAGAPP AARAGYAHFL NVQGRTLYDV ILYGLQEHSE VSGFLLECDS SVQGALQKHL ALYRIRRKVT VEPHPELRVW AVLPSSPEAC GAASLQERAG AAAILIRDPR TARMGWRLLT QDEGPALVPG GRLGDLWDYH QHRYLQGVPE GVRDLPPGVA LPLESNLAFM NGVSFTKGCY IGQELTARTH HMGVIRKRLF PVRFLDPLPT SGITPGATVL TASGQTVGKF RAGQGNVGLA LLWSEKIKGP LHIRASEGAQ VALAASVPDW WPTVSK
蛋白標(biāo)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(chǎn)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(fù)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(zhì)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(chǎn)品評價

靶點詳情

功能：

Involved in the maturation of mitochondrial 4Fe-4S proteins functioning late in the iron-sulfur cluster assembly pathway.
基因功能參考文獻(xiàn)：
1. a patient with infantile-onset optic atrophy and asymptomatic white matter involvement, thus broadening the phenotypic spectrum of biallelic IBA57 mutations. PMID: 30258207
2. Description of four unrelated patients carrying novel mutations in IBA57; study expands the array of the genotypic variation of IBA57 and delineates the leukodystrophic pattern of IBA57 deficient patients PMID: 27785568
3. Findings reinforce the suggested specific function of IBA57 in mitochondrial [4Fe-4S] protein maturation and provide additional evidence for its role in hereditary spastic paraplegia PMID: 25609768
4. Mutation of the iron-sulfur cluster assembly gene IBA57 causes severe myopathy and encephalopathy. PMID: 23462291
相關(guān)疾病：

Multiple mitochondrial dysfunctions syndrome 3 (MMDS3); Spastic paraplegia 74, autosomal recessive (SPG74)
亞細(xì)胞定位：

Mitochondrion.
蛋白家族：

GcvT family, CAF17 subfamily
組織特異性：

Expressed in skin fibroblasts and skeletal muscle (at protein level).
數(shù)據(jù)庫鏈接：

HGNC: 27302

OMIM: 615316

KEGG: hsa:200205

STRING: 9606.ENSP00000355672

UniGene: Hs.237017