UniProtKB - Q2LAM0 (FA2H_RAT)
Fatty acid 2-hydroxylase
Fa2h
Functioni
Catalyzes the hydroxylation of free fatty acids at the C-2 position to produce 2-hydroxy fatty acids, which are building blocks of sphingolipids and glycosphingolipids common in neural tissue and epidermis (Probable). FA2H is stereospecific for the production of (R)-2-hydroxy fatty acids (By similarity).
Plays an essential role in the synthesis of galactosphingolipids of the myelin sheath (PubMed:17901466).
Responsible for the synthesis of sphingolipids and glycosphingolipids involved in the formation of epidermal lamellar bodies critical for skin permeability barrier (By similarity).
Participates in the synthesis of glycosphingolipids and a fraction of type II wax diesters in sebaceous gland, specifically regulating hair follicle homeostasis. Involved in the synthesis of sphingolipids of plasma membrane rafts, controlling lipid raft mobility and trafficking of raft-associated proteins (By similarity).
By similarity1 Publication1 PublicationCatalytic activityi
- a 1,2-saturated fatty acid + 2 Fe(II)-[cytochrome b5] + 2 H+ + O2 = a (R)-2-hydroxy fatty acid + 2 Fe(III)-[cytochrome b5] + H2OBy similarity1 PublicationThis reaction proceeds in the forwardBy similarity direction.
- 2 Fe(II)-[cytochrome b5] + 2 H+ + hexadecanoate + O2 = (R)-2-hydroxyhexadecanoate + 2 Fe(III)-[cytochrome b5] + H2OBy similarityThis reaction proceeds in the forwardBy similarity direction.
- 2 Fe(II)-[cytochrome b5] + 2 H+ + O2 + octadecanoate = (R)-2-hydroxyoctadecanoate + 2 Fe(III)-[cytochrome b5] + H2OBy similarityThis reaction proceeds in the forwardBy similarity direction.
- docosanoate + 2 Fe(II)-[cytochrome b5] + 2 H+ + O2 = 2-hydroxydocosanoate + 2 Fe(III)-[cytochrome b5] + H2OBy similarityThis reaction proceeds in the forwardBy similarity direction.
- 2 Fe(II)-[cytochrome b5] + 2 H+ + O2 + tetracosanoate = (R)-2-hydroxytetracosanoate + 2 Fe(III)-[cytochrome b5] + H2OBy similarity1 Publication
Cofactori
: fatty acid metabolism Pathwayi
This protein is involved in the pathway fatty acid metabolism, which is part of Lipid metabolism.1 PublicationView all proteins of this organism that are known to be involved in the pathway fatty acid metabolism and in Lipid metabolism.
Pathwayi: galactosylceramide biosynthesis
This protein is involved in the pathway galactosylceramide biosynthesis, which is part of Sphingolipid metabolism.1 PublicationView all proteins of this organism that are known to be involved in the pathway galactosylceramide biosynthesis and in Sphingolipid metabolism.
Sites
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Metal bindingi | 43 | Iron (heme axial ligand)PROSITE-ProRule annotation | 1 | |
Metal bindingi | 69 | Iron (heme axial ligand)PROSITE-ProRule annotation | 1 | |
Metal bindingi | 234 | Zinc 1; via tele nitrogenBy similarity | 1 | |
Metal bindingi | 239 | Zinc 1; via tele nitrogenBy similarity | 1 | |
Metal bindingi | 257 | Zinc 1; via tele nitrogenBy similarity | 1 | |
Metal bindingi | 260 | Zinc 2; via tele nitrogenBy similarity | 1 | |
Metal bindingi | 261 | Zinc 1; via tele nitrogenBy similarity | 1 | |
Metal bindingi | 315 | Zinc 2; via tele nitrogenBy similarity | 1 | |
Metal bindingi | 319 | Zinc 2; via tele nitrogenBy similarity | 1 | |
Metal bindingi | 336 | Zinc 2; via tele nitrogenBy similarity | 1 | |
Metal bindingi | 339 | Zinc 1; via tele nitrogenBy similarity | 1 | |
Metal bindingi | 340 | Zinc 2; via tele nitrogenBy similarity | 1 |
GO - Molecular functioni
- fatty acid alpha-hydroxylase activity Source: UniProtKB
- heme binding Source: InterPro
- iron ion binding Source: InterPro
GO - Biological processi
- central nervous system myelin maintenance Source: RGD
- ceramide biosynthetic process Source: UniProtKB
- establishment of skin barrier Source: UniProtKB
- fatty acid biosynthetic process Source: UniProtKB-KW
- fatty acid metabolic process Source: RGD
- galactosylceramide biosynthetic process Source: UniProtKB
- glucosylceramide biosynthetic process Source: UniProtKB
- lipid modification Source: RGD
- peripheral nervous system myelin maintenance Source: RGD
- plasma membrane raft organization Source: UniProtKB
- regulation of cell population proliferation Source: RGD
- regulation of hair cycle Source: RGD
- sebaceous gland cell differentiation Source: RGD
Keywordsi
Molecular function | Oxidoreductase |
Biological process | Fatty acid biosynthesis, Fatty acid metabolism, Lipid biosynthesis, Lipid metabolism, Sphingolipid metabolism |
Ligand | Heme, Iron, Metal-binding, Zinc |
Enzyme and pathway databases
Reactomei | R-RNO-1660661, Sphingolipid de novo biosynthesis |
UniPathwayi | UPA00199 UPA00787 |
Chemistry databases
SwissLipidsi | SLP:000001975 |
Names & Taxonomyi
Protein namesi | Recommended name: Fatty acid 2-hydroxylase1 Publication (EC:1.14.18.-By similarity1 Publication)Alternative name(s): Fatty acid alpha-hydroxylase1 Publication |
Gene namesi | |
Organismi | Rattus norvegicus (Rat) |
Taxonomic identifieri | 10116 [NCBI] |
Taxonomic lineagei | Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Glires › Rodentia › Myomorpha › Muroidea › Muridae › Murinae › Rattus |
Proteomesi |
|
Organism-specific databases
RGDi | 1310347, Fa2h |
Subcellular locationi
Endoplasmic reticulum
- Endoplasmic reticulum membrane By similarity; Multi-pass membrane protein By similarity
- Microsome membrane By similarity; Multi-pass membrane protein By similarity
Endoplasmic reticulum
- endoplasmic reticulum Source: RGD
- endoplasmic reticulum membrane Source: UniProtKB-SubCell
Other locations
- integral component of membrane Source: UniProtKB-KW
- membrane Source: UniProtKB
Topology
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Transmembranei | 168 – 188 | HelicalSequence analysisAdd BLAST | 21 | |
Transmembranei | 213 – 233 | HelicalSequence analysisAdd BLAST | 21 | |
Transmembranei | 268 – 288 | HelicalSequence analysisAdd BLAST | 21 | |
Transmembranei | 290 – 310 | HelicalSequence analysisAdd BLAST | 21 |
Keywords - Cellular componenti
Endoplasmic reticulum, Membrane, MicrosomePTM / Processingi
Molecule processing
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
ChainiPRO_0000312352 | 1 – 372 | Fatty acid 2-hydroxylaseAdd BLAST | 372 |
Proteomic databases
PaxDbi | Q2LAM0 |
Expressioni
Tissue specificityi
Developmental stagei
Inductioni
Gene expression databases
Bgeei | ENSRNOG00000018950, Expressed in stomach and 18 other tissues |
Genevisiblei | Q2LAM0, RN |
Family & Domainsi
Domains and Repeats
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Domaini | 8 – 86 | Cytochrome b5 heme-bindingPROSITE-ProRule annotationAdd BLAST | 79 | |
Domaini | 219 – 361 | Fatty acid hydroxylaseSequence analysisAdd BLAST | 143 |
Domaini
Sequence similaritiesi
Keywords - Domaini
Transmembrane, Transmembrane helixPhylogenomic databases
eggNOGi | KOG0537, Eukaryota KOG0539, Eukaryota |
GeneTreei | ENSGT00390000002142 |
HOGENOMi | CLU_034756_2_0_1 |
InParanoidi | Q2LAM0 |
OMAi | HFADYEN |
OrthoDBi | 1049908at2759 |
PhylomeDBi | Q2LAM0 |
TreeFami | TF314955 |
Family and domain databases
Gene3Di | 3.10.120.10, 1 hit |
InterProi | View protein in InterPro IPR001199, Cyt_B5-like_heme/steroid-bd IPR036400, Cyt_B5-like_heme/steroid_sf IPR018506, Cyt_B5_heme-BS IPR006694, Fatty_acid_hydroxylase IPR014430, Scs7 |
PANTHERi | PTHR12863, PTHR12863, 1 hit |
Pfami | View protein in Pfam PF00173, Cyt-b5, 1 hit PF04116, FA_hydroxylase, 1 hit |
PIRSFi | PIRSF005149, IPC-B_HD, 1 hit |
PRINTSi | PR00363, CYTOCHROMEB5 |
SMARTi | View protein in SMART SM01117, Cyt-b5, 1 hit |
SUPFAMi | SSF55856, SSF55856, 1 hit |
PROSITEi | View protein in PROSITE PS00191, CYTOCHROME_B5_1, 1 hit PS50255, CYTOCHROME_B5_2, 1 hit |
i Sequence
Sequence statusi: Complete.
10 20 30 40 50
MAPAPPPAAS FTSAEVQRRL AAGACWVRRG ASLYDLTGFV RHHPGGEQLL
60 70 80 90 100
LARAGQDISA DLDGPPHKHS DNARRWLEQY YVGELRADPQ DPTENGAGAP
110 120 130 140 150
AETQKTDAAI EPQFKVVDWD KDLVDWQKPL LWQVGHLGEK YDEWVHQPVA
160 170 180 190 200
RPIRLFHSDL IEAFSKTVWY SVPIIWVPLV LYLSWSYYRT LTQDNIRLFA
210 220 230 240 250
SFTRDYSLVV PESVFIGLFV LGMLIWTLVE YLIHRFLFHM KPPSNSHYLI
260 270 280 290 300
MLHFVMHGQH HKAPFDGSRL VFPPVPASVV VAFFYVFLRL ILPEAVAGIL
310 320 330 340 350
FAGGLLGYVL YDMTHYYLHF GSPHKGSYLY NMKAHHVKHH FEYQKSGFGI
360 370
STKLWDYFFH TLIPEEADPK MQ
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | DQ339484 mRNA Translation: ABC71132.1 CO396956 mRNA No translation available. |
RefSeqi | NP_001129055.1, NM_001135583.1 |
Genome annotation databases
Ensembli | ENSRNOT00000025625; ENSRNOP00000025625; ENSRNOG00000018950 |
GeneIDi | 307855 |
KEGGi | rno:307855 |
UCSCi | RGD:1310347, rat |
Similar proteinsi
Cross-referencesi
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | DQ339484 mRNA Translation: ABC71132.1 CO396956 mRNA No translation available. |
RefSeqi | NP_001129055.1, NM_001135583.1 |
3D structure databases
SMRi | Q2LAM0 |
ModBasei | Search... |
Protein-protein interaction databases
STRINGi | 10116.ENSRNOP00000025625 |
Chemistry databases
SwissLipidsi | SLP:000001975 |
Proteomic databases
PaxDbi | Q2LAM0 |
Genome annotation databases
Ensembli | ENSRNOT00000025625; ENSRNOP00000025625; ENSRNOG00000018950 |
GeneIDi | 307855 |
KEGGi | rno:307855 |
UCSCi | RGD:1310347, rat |
Organism-specific databases
CTDi | 79152 |
RGDi | 1310347, Fa2h |
Phylogenomic databases
eggNOGi | KOG0537, Eukaryota KOG0539, Eukaryota |
GeneTreei | ENSGT00390000002142 |
HOGENOMi | CLU_034756_2_0_1 |
InParanoidi | Q2LAM0 |
OMAi | HFADYEN |
OrthoDBi | 1049908at2759 |
PhylomeDBi | Q2LAM0 |
TreeFami | TF314955 |
Enzyme and pathway databases
UniPathwayi | UPA00199 UPA00787 |
Reactomei | R-RNO-1660661, Sphingolipid de novo biosynthesis |
Miscellaneous databases
PROi | PR:Q2LAM0 |
Gene expression databases
Bgeei | ENSRNOG00000018950, Expressed in stomach and 18 other tissues |
Genevisiblei | Q2LAM0, RN |
Family and domain databases
Gene3Di | 3.10.120.10, 1 hit |
InterProi | View protein in InterPro IPR001199, Cyt_B5-like_heme/steroid-bd IPR036400, Cyt_B5-like_heme/steroid_sf IPR018506, Cyt_B5_heme-BS IPR006694, Fatty_acid_hydroxylase IPR014430, Scs7 |
PANTHERi | PTHR12863, PTHR12863, 1 hit |
Pfami | View protein in Pfam PF00173, Cyt-b5, 1 hit PF04116, FA_hydroxylase, 1 hit |
PIRSFi | PIRSF005149, IPC-B_HD, 1 hit |
PRINTSi | PR00363, CYTOCHROMEB5 |
SMARTi | View protein in SMART SM01117, Cyt-b5, 1 hit |
SUPFAMi | SSF55856, SSF55856, 1 hit |
PROSITEi | View protein in PROSITE PS00191, CYTOCHROME_B5_1, 1 hit PS50255, CYTOCHROME_B5_2, 1 hit |
MobiDBi | Search... |
Entry informationi
Entry namei | FA2H_RAT | |
Accessioni | Q2LAM0Primary (citable) accession number: Q2LAM0 | |
Entry historyi | Integrated into UniProtKB/Swiss-Prot: | December 4, 2007 |
Last sequence update: | December 4, 2007 | |
Last modified: | February 23, 2022 | |
This is version 101 of the entry and version 2 of the sequence. See complete history. | ||
Entry statusi | Reviewed (UniProtKB/Swiss-Prot) | |
Annotation program | Chordata Protein Annotation Program |
Miscellaneousi
Keywords - Technical termi
Reference proteomeDocuments
- PATHWAY comments
Index of metabolic and biosynthesis pathways - SIMILARITY comments
Index of protein domains and families