Anti-CYP27B1抗体The cytochrome P450 proteins are monooxygenases that catalyze many reactions involved in drug metabolism and synthesis of cholesterol,

产品编号 yb-14146R
英文名称 Anti-CYP27B1抗体
中文名称 细胞色素P450 27B1抗体
别    名 1alpha(OH)ase; 25-hydroxyvitamin D(3) 1-alpha-hydroxylase; 25-hydroxyvitamin D-1 alpha hydroxylase; 25-OHD-1 alpha-hydroxylase; Calcidiol 1-monooxygenase; CP27B_HUMAN; CP2B; CYP1; CYP1ALPHA; CYP27B; Cyp27b1; Cytochrome p450 27B1; Cytochrome p450 27B13; Cytochrome P450 family 27 subfamily B polypeptide 1; Cytochrome P450 subfamily XXVIIB (25-hydroxyvitamin D-1-alpha-hydroxylase) polypeptide 1; Cytochrome P450 subfamily XXVIIB polypeptide 1; Cytochrome P450C1 alpha; Cytochrome P450VD1-alpha; mitochondrial; P450C1 alpha; P450c1; P450C1-alpha; P450VD1-alpha; PDDR; VD3 1A hydroxylase; VDD1; VDDR; VDDR I; VDDRI; VDR.
 Anti-CYP27B1抗体
说 明 书 0.1ml  0.2ml  
研究领域 细胞生物  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Dog, Pig, Cow, Horse, Sheep, 
产品应用 WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 57kDa
细胞定位 细胞膜 
性    状 Lyophilized or Liquid
浓    度 1mg/1ml
免 疫 原 KLH conjugated synthetic peptide derived from human CYP27B1
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 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.
 
PubMed PubMed
产品介绍 background:
The cytochrome P450 proteins are monooxygenases that catalyze many reactions involved in drug metabolism and synthesis of cholesterol, steroids and other lipids. P450 enzymes are classified into subfamilies based on their sequence similarities. CYP27B1, a 508-amino acid protein that belongs to the XXVIIB subfamily of the cytochrome P450 family, localizes to the mitochondrion and is expressed in the kidney. The CYP27B1 protein catalyzes the conversion of 25-hydroxyvitamin D3 (25(OH)D) to 1-alpha,25-dihydroxyvitamin D3 (1,25(OH)2D) and functions in calcium metabolism, normal bone growth, and tissue differentiation. Mutations in the gene which encodes for CYP27B1 cause vitamin D-dependent rickets type 1 (VDDR-1), also designated pseudovitamin D deficiency rickets (PDDR), an autosomal recessive disease characterized by early onset of rickets with hypocalcemia and muscle weakness.

Function:
Catalyzes the conversion of 25-hydroxyvitamin D3 (25(OH)D) to 1-alpha,25-dihydroxyvitamin D3 (1,25(OH)2D) plays an important role in normal bone growth, calcium metabolism, and tissue differentiation.

Subcellular Location:
Mitochondrion membrane.

Tissue Specificity:
Kidney.

DISEASE:
Defects in CYP27B1 are the cause of rickets vitamin D-dependent type 1A (VDDR1A) [MIM:264700]; also known as pseudovitamin D deficiency rickets (PDDR). A disorder caused by a selective deficiency of the active form of vitamin D (1,25-dihydroxyvitamin D3) and resulting in defective bone mineralization and clinical features of rickets.