Reactive species
Human;Mouse;Rat
Antibody type
Polyclonal Antibody
Immunogen
Synthesized peptide derived from human FA2H. at AA range: 101-150
Specificity
FA2H Polyclonal Antibody detects endogenous levels of FA2H
Constitute
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Source
Polyclonal, Rabbit,IgG
Dilution rate
WB 1:500-2000, ELISA 1:10000-20000
Purification process
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Other name
Fatty acid 2-hydroxylase (EC 1.-.-.-) (Fatty acid alpha-hydroxylase)
Background
This gene encodes a protein that catalyzes the synthesis of 2-hydroxysphingolipids, a subset of sphingolipids that contain 2-hydroxy fatty acids. Sphingolipids play roles in many cellular processes and their structural diversity arises from modification of the hydrophobic ceramide moiety, such as by 2-hydroxylation of the N-acyl chain, and the existence of many different head groups. Mutations in this gene have been associated with leukodystrophy dysmyelinating with spastic paraparesis with or without dystonia.[provided by RefSeq, Mar 2010],
Function
cofactor:Iron.,disease:Defects in FA2H are the cause of leukodystrophy dysmyelinating with spastic paraparesis with or without dystonia (DLDSP) [MIM:612443]. The disorder consists of a progressive neurologic disease manifested by spasticity, disordered tonicity of muscle, and white matter degeneration.,domain:The histidine box domains may contain the active site and/or be involved in metal ion binding.,function:Required for alpha-hydroxylation of free fatty acids and the formation of alpha-hydroxylated sphingolipids.,induction:Up-regulated during keratinocyte differentiation.,similarity:Belongs to the SCS7 family.,similarity:Contains 1 cytochrome b5 heme-binding domain.,tissue specificity:Detected in differentiating cultured keratinocytes (at protein level). Detected in epidermis and cultured keratinocytes. Highly expressed in brain and colon. Detected at lower levels in testis, prostate
