FA2H Rabbit Polyclonal Antibody

• 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], 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, pancreas and kidney.

• Product Name Alternative:

  Fatty acid 2-hydroxylase (EC 1.-.-.-) (Fatty acid alpha-hydroxylase)

• Gene Name:

  FA2H

• Gene ID:

  79152

• Swiss Prot:

  Q7L5A8

• Host:

  Rabbit

• Reactivity:

  Human, Mouse, Rat

• Clonality:

  Polyclonal

• Conjugation:

  Unconjugated

• Applications:

  WB, ELISA

• Purification:

  Affinity purification

• Dilution:

  WB 1:500-1:2000, ELISA 1:10000-1:20000

• Form:

  Liquid

• Buffer:

  Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N.

• Modification:

  Unmodified

• Molecular Weight:

  55kDa

• Storage Conditions:

  Store at 4°C short term. Aliquot and store at -20°C for 12 months. Avoid freeze/thaw cycles.

• Isotype:

  IgG