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Rabbit Anti-MPI/Mannose Phosphate Isomerase/BF350 Conjugated antibody (bs-18654R-BF350)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產(chǎn)品編號 bs-18654R-BF350
英文名稱 Rabbit Anti-MPI/Mannose Phosphate Isomerase/BF350 Conjugated antibody
中文名稱 BF350標記的磷酸甘露糖異構(gòu)酶抗體
別    名 PMI1; CDG1B; FLJ39201; Mannose 6 phosphate isomerase; Mannose-6-phosphate isomerase; MANNOSEPHOSPHATE ISOMERASE; MGC94106; MPI; MPI_HUMAN; Phosphohexomutase; phosphomannose isomerase 1; Phosphomannose isomerase; PMI.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領(lǐng)域 腫瘤  細胞生物  免疫學  信號轉(zhuǎn)導  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) (predicted: Human, Mouse, Rat, Pig, Cow, Horse, Sheep, )
產(chǎn)品應(yīng)用 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 46kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human MPI/Mannose Phosphate Isomerase
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol
保存條件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
產(chǎn)品介紹 background:
Phosphomannose isomerase catalyzes the interconversion of fructose-6-phosphate and mannose-6-phosphate and plays a critical role in maintaining the supply of D-mannose derivatives, which are required for most glycosylation reactions. Mutations in the MPI gene were found in patients with carbohydrate-deficient glycoprotein syndrome, type Ib. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jan 2014]

Function:
Involved in the synthesis of the GDP-mannose and dolichol-phosphate-mannose required for a number of critical mannosyl transfer reactions.

Subcellular Location:
Cytoplasm.

Tissue Specificity:
Expressed in all tissues, but more abundant in heart, brain and skeletal muscle.

DISEASE:
Defects in MPI are the cause of congenital disorder of glycosylation type 1B (CDG1B) [MIM:602579]; also known as carbohydrate-deficient glycoprotein syndrome type Ib (CDGS1B). Congenital disorders of glycosylation are metabolic deficiencies in glycoprotein biosynthesis that usually cause severe mental and psychomotor retardation. They are characterized by under-glycosylated serum glycoproteins. CDG1B is clinically characterized by protein-losing enteropathy.

Similarity:
Belongs to the mannose-6-phosphate isomerase type 1 family.

Database links:

Entrez Gene: 4351 Human

Entrez Gene: 513586 Cow

Entrez Gene: 110119 Mouse

Entrez Gene: 300741 Rat

Omim: 154550 Human

SwissProt: Q3SZI0 Cow

SwissProt: P34949 Human

SwissProt: Q924M7 Mouse

SwissProt: Q68FX1 Rat

Unigene: 75694 Human

Unigene: 247218 Mouse

Unigene: 44246 Rat



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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