fa2h
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  • Official Full Name
  • fatty acid 2-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.
  • Synonyms
  • FA2H; fatty acid 2-hydroxylase; fatty acid hydroxylase domain containing 1 , FAXDC1, spastic paraplegia 35 (autosomal recessive) , SPG35; FAAH; fatty acid hydroxylase; FLJ25287; FA2H_HUMAN; FAH1; Fatty acid 2 hydroxylase; Fatty acid alpha hydroxylase; Fatty acid alpha-hydroxylase; Fatty acid hydroxylase domain containing 1; FAXDC1; SCS7; spastic paraplegia 35 (autosomal recessive); SPG35
Cat.#:FA2H-12628HTag:His
Source (Host):E. coliSpecies:Human
Product nameRecombinant Human FA2H, His-tagged
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Cat.#:FA2H-891HTag:MYC/DDK
Source (Host):HEK293Species:Human
Product nameRecombinant Human FA2H, MYC/DDK-tagged
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Cat.#:FA2H-893HTag:GST
Source (Host):Wheat germSpecies:Human
Product nameRecombinant Human FA2H, GST-tagged
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Cat.#:FA2H-5419MTag:His
Source (Host):Mammalian CellsSpecies:Mouse
Product nameRecombinant Mouse FA2H Protein
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Cat.#:FA2H-2182RTag:His
Source (Host):Mammalian CellsSpecies:Rat
Product nameRecombinant Rat FA2H Protein
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Cat.#:FA2H-501CTag:His
Source (Host):Mammalian CellsSpecies:Cynomolgus Monkey
Product nameRecombinant Cynomolgus FA2H Protein, His-tagged
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Cat.#:FA2H-1534RTag:His
Source (Host):Mammalian CellsSpecies:Rhesus Macaque
Product nameRecombinant Rhesus monkey FA2H Protein, His-tagged
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Cat.#:FA2H-4375ZTag:His
Source (Host):Mammalian CellsSpecies:Zebrafish
Product nameRecombinant Zebrafish FA2H
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Cat.#:FA2H-6481HCLTag:
Source (Host):Species:Human
Product nameRecombinant Human FA2H 293 Cell Lysate
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Involved Pathway

FA2H involved in several pathways and played different roles in them. We selected most pathways FA2H participated on our site, such as Metabolism, Metabolism of lipids and lipoproteins, Sphingolipid de novo biosynthesis, which may be useful for your reference. Also, other proteins which involved in the same pathway with FA2H were listed below. Creative BioMart supplied nearly all the proteins listed, you can search them on our site.
Pathway Name
Pathway Related Protein
Metabolism
UGT1B1; NAGS; NUDT18; CYGB2; UGT1B3; CYP2F1; MED29; EPM2A; CERS4A; DHODH
Metabolism of lipids and lipoproteins
SLC25A20; TIAM2; AKR1C2; SACM1L; FABP1B.1; TMEM195; GLTP; CYP46A1; PLIN2; ALOX5B.3
Sphingolipid de novo biosynthesis
PPM1LB; PPM1LA; VAPB; CERS3B; FA2H; SPTLC1; OSBP; GDF1; CERS5; PPM1L
Sphingolipid metabolism
ARSH; SGPP1; PPAP2C; CERK; ARSJ; SGMS1; GALCA; DEGS1; SPTLC2; ARSA
fatty acid alpha-oxidation III
FA2H

Protein Function

FA2H has several biochemical functions, for example, fatty acid alpha-hydroxylase activity, heme binding, iron ion binding. Some of the functions are cooperated with other proteins, some of the functions could acted by FA2H itself. We selected most functions FA2H had, and list some proteins which have the same functions with FA2H. You can find most of the proteins on our site.
Function
Related Protein
Function fatty acid alpha-hydroxylase activity
Related Protein FA2H
Function heme binding
Related Protein CYP1D1; CAT; CYP2AA2; ABCB6; CYP4A14; CYP2S1; NGB; CYP2K22; HRG; CYP2X9
Function iron ion binding
Related Protein HBD; CYP2D26; CYP4A12A; CYP26B1; CYP2H1; CYP2K22; CH25HL2; PLOD2; HIF1AN; JMJD6

FA2H Related Articles

Pedroso, JL; Handfas, BW; et al. Fatty acid 2-hydroxylase deficiency Clinical features and brain iron accumulation. NEUROLOGY 84:960-961(2015).
Liao, X; Luo, Y; et al. SPG35 contributes to the second common subtype of AR-HSP in China: frequency analysis and functional characterization of FA2H gene mutations. CLINICAL GENETICS 87:85-89(2015).