Recombinant Mouse Propionyl-CoA carboxylase alpha chain, mitochondrial (Pcca)

中文名稱：

小鼠Pcca重組蛋白
貨號：

CSB-YP849688MO
說明書：

MSDS Datasheet
規格：
來源：

Yeast
其他：

中文名稱：

小鼠Pcca重組蛋白
貨號：

CSB-EP849688MO
說明書：

MSDS Datasheet
規格：
來源：

E.coli
其他：

中文名稱：

小鼠Pcca重組蛋白
貨號：

CSB-EP849688MO-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：

小鼠Pcca重組蛋白
貨號：

CSB-BP849688MO
說明書：

MSDS Datasheet
規格：
來源：

Baculovirus
其他：

中文名稱：

小鼠Pcca重組蛋白
貨號：

CSB-MP849688MO
說明書：

MSDS Datasheet
規格：
來源：

Mammalian cell
其他：

產品詳情
產品評價
靶點詳情

產品詳情

純度：

>85% (SDS-PAGE)
基因名：

Pcca
Uniprot No.：

Q91ZA3
別名：

Pcca; Propionyl-CoA carboxylase alpha chain; mitochondrial; PCCase subunit alpha; EC 6.4.1.3; Propanoyl-CoA:carbon dioxide ligase subunit alpha
種屬：

Mus musculus (Mouse)
蛋白長度：

Full Length of Mature Protein
表達區域：

49-724
氨基酸序列

SV EYEPKEKTFD KILIANRGEI ACRVIKTCKK MGIKTVAIHS DVDASSVHVK MADEAVCVGP APTSKSYLNM DAIMEAIKKT RAQAVHPGYG FLSENKEFAK RLAAEDVTFI GPDTHAIQAM GDKIESKLLA KRAKVNTIPG FDGVVKDADE AVRIAREIGY PVMIKASAGG GGKGMRIAWD DEETRDGFRF SSQEAASSFG DDRLLIEKFI DNPRHIEIQV LGDKHGNALW LNERECSIQR RNQKVVEEAP SIFLDPETRQ AMGEQAVALA KAVKYSSAGT VEFLVDSQKN FYFLEMNTRL QVEHPVTECI TGLDLVQEMI LVAKGYPLRH KQEDIPISGW AVECRVYAED PYKSFGLPSI GRLSQYQEPI HLPGVRVDSG IQPGSDISIY YDPMISKLVT YGSDRAEALK RMEDALDNYV IRGVTHNIPL LREVIINTRF VKGDISTKFL SDVYPDGFKG HTLTLSERNQ LLAIASSVFV ASQLRAQRFQ EHSRVPVIRP DVAKWELSVK LHDEDHTVVA SNNGPAFTVE VDGSKLNVTS TWNLASPLLS VNVDGTQRTV QCLSREAGGN MSIQFLGTVY KVHILTKLAA ELNKFMLEKV PKDTSSTLCS PMPGVVVAVS VKPGDMVAEG QEICVIEAMK MQNSMTAGKM GKVKLVHCKA GDTVGEGDLL VELE
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：

This is one of the 2 subunits of the biotin-dependent propionyl-CoA carboxylase (PCC), a mitochondrial enzyme involved in the catabolism of odd chain fatty acids, branched-chain amino acids isoleucine, threonine, methionine, and valine and other metabolites. Propionyl-CoA carboxylase catalyzes the carboxylation of propionyl-CoA/propanoyl-CoA to D-methylmalonyl-CoA/(S)-methylmalonyl-CoA. Within the holoenzyme, the alpha subunit catalyzes the ATP-dependent carboxylation of the biotin carried by the biotin carboxyl carrier (BCC) domain, while the beta subunit then transfers the carboxyl group from carboxylated biotin to propionyl-CoA. Propionyl-CoA carboxylase also significantly acts on butyryl-CoA/butanoyl-CoA, which is converted to ethylmalonyl-CoA/(2S)-ethylmalonyl-CoA. Other alternative minor substrates include (2E)-butenoyl-CoA/crotonoyl-CoA.
基因功能參考文獻：
1. we have investigated mitochondrial function, superoxide anion and H2O2 levels and oxidative damage in relevant tissues of the hypomorphic mice(knockout mouse model for the Pcca gene), to gain insight into the pathophysiological processes contributing to the multiorgan complications observed in propionic acidemia PMID: 27083476
2. A single intravenous injection of adeno-associated virus 2/8 or AAVrh10 expressing PCCA into propionic academia hypomorphic mice decreased systemic propionylcarnitine and methyl citrate for up to 1.5 years. PMID: 25654275
相關疾?。?/div>
Propionic acidemia due to recessively inherited deficiency of PCCase activity often causes life-threatening ketosis and acidosis.
亞細胞定位：

Mitochondrion matrix.
數據庫鏈接：

KEGG: mmu:110821

STRING: 10090.ENSMUSP00000038763

UniGene: Mm.23876