Rabbit Anti-CYP27B1/FITC Conjugated antibody

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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 p4

Cat:

SL14146R-FITC

Species Reactivity：

Mouse,(predicted: Human,Rat,Dog,Pig,Cow,Horse,Sheep,)

Immunogen：

KLH conjugated synthetic peptide derived from human CYP27B1

Format：

Lyophilized or Liquid

Storage instructions：

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 ant

Buffer：

0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

Concentration：

1mg/ml

Clonality：

Polyclonal

Isotype：

IgG

Applications：

ICC=1:50-200IF=1:50-200not yet tested in other applications.optimal dilutions/concentrations should be determined by the end user.

Host：

Rabbit

Calculated MW：

57kDa

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Unit:

Price: $596.00

Product PDFs

Datasheet: Down Datasheet

Other Information
Citations
Protein Attributes
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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.

Similarity:
Belongs to the cytochrome P450 family.

Database links:

Entrez Gene: 1594 Human

Omim: 609506 Human

SwissProt: O15528 Human

Unigene: 524528 Human

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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