Anti-Cytochrome P450 7B1 [M17-P3F2]

Defects in CYP7B1 are the cause of spastic paraplegia autosomal recessive type 5A (SPG5A) [MIM:270800].

Contributors

Inventor Institute
Ayham Alnabulsi Vertebrate Antibodies Limited
SKU: 152117
Product description: Defects in CYP7B1 are the cause of spastic paraplegia autosomal recessive type 5A (SPG5A) [MIM:270800]. Spastic paraplegia is a neurodegenerative disorder characterized by a slow, gradual, progressive weakness and spasticity of the lower limbs. Rate of progression and the severity of symptoms are quite variable. Initial symptoms may include difficulty with balance, weakness and stiffness in the legs, muscle spasms, and dragging the toes when walking. In some forms of the disorder, bladder symptoms (such as incontinence) may appear, or the weakness and stiffness may spread to other parts of the body. Defects in CYP7B1 are the cause of congenital bile acid synthesis defect type 3 (CBAS3) [MIM:613812]. Clinical features include severe cholestasis, cirrhosis and liver synthetic failure. Hepatic microsomal oxysterol 7-alpha-hydroxylase activity is undetectable.
Conjugation: Unconjugated
Isotype: IgG1 lambda
Immunogen: Ovalbumin-conjugated synthetic peptide IQYPDSDVL (C-terminal sequence)
Myeloma used: P3X63Ag8.653
Cat. #: 152117
Tool sub type: Primary antibody
Unit size: 100 ug
Research Fields: Cancer; Cell signaling and signal transduction; Metabolism; Neurobiology; Tissue-specific biology
Application: ELISA ; IHC ; WB
Target: Cytochrome P450, family 7, subfamily B, polypeptide 1 (CYP7B1)
Reactivity: Human
Clone: M17-P3F2
Host: Mouse
Class: Monoclonal
Target background: Defects in CYP7B1 are the cause of spastic paraplegia autosomal recessive type 5A (SPG5A) [MIM:270800]. Spastic paraplegia is a neurodegenerative disorder characterized by a slow, gradual, progressive weakness and spasticity of the lower limbs. Rate of progression and the severity of symptoms are quite variable. Initial symptoms may include difficulty with balance, weakness and stiffness in the legs, muscle spasms, and dragging the toes when walking. In some forms of the disorder, bladder symptoms (such as incontinence) may appear, or the weakness and stiffness may spread to other parts of the body. Defects in CYP7B1 are the cause of congenital bile acid synthesis defect type 3 (CBAS3) [MIM:613812]. Clinical features include severe cholestasis, cirrhosis and liver synthetic failure. Hepatic microsomal oxysterol 7-alpha-hydroxylase activity is undetectable.
Format: Liquid
Concentration: 1 mg/ml
Storage buffer: PBS with 0.02% azide
Storage conditions: -15° C to -25° C
Shipping conditions: Dry ice
References:

Characterisation of the oxysterol metabolising enzyme pathway in mismatch repair proficient and deficient colorectal cancer.

Swan et al. 2016. Oncotarget. :. PMID: 27341022.

Images

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Immunohistochemistry was performed on formalin-fixed; paraffin-embedded tissue sections and showed positive cytoplasmic immunostaining in normal liver (A) which is expected as CYP7B1 is involved in bile acid synthesis. Primary colorectal (B) and metastatic colorectal tumors (C) showed stronger staining compared to normal colon mucosa (D).

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