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Protein

Alkaline ceramidase 1

Gene

ACER1

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -Experimental evidence at transcript leveli

Functioni

Hydrolyzes the sphingolipid ceramide into sphingosine and free fatty acid at an optimal pH of 8.0. Has a highly restricted substrate specificity for the natural stereoisomer of ceramide with D-erythro-sphingosine but not D-ribo-phytosphingosine or D-erythro-dihydrosphingosine as a backbone. May have a role in regulating the levels of bioactive lipids ceramide and sphingosine 1-phosphate, as well as complex sphingolipids (By similarity).By similarity

Catalytic activityi

N-acylsphingosine + H2O = a carboxylate + sphingosine.

Enzyme regulationi

Inhibited by sphingosine.By similarity

GO - Molecular functioni

  • ceramidase activity Source: UniProtKB-EC
  • dihydroceramidase activity Source: BHF-UCL
  • N-acylsphingosine amidohydrolase activity Source: BHF-UCL

GO - Biological processi

  • cell differentiation Source: BHF-UCL
  • cellular response to calcium ion Source: BHF-UCL
  • ceramide catabolic process Source: BHF-UCL
  • epidermis development Source: BHF-UCL
  • keratinocyte differentiation Source: BHF-UCL
  • regulation of lipid metabolic process Source: Ensembl
  • response to alkaline pH Source: BHF-UCL
  • sphingolipid biosynthetic process Source: BHF-UCL
  • sphingolipid metabolic process Source: BHF-UCL
  • sphingosine biosynthetic process Source: BHF-UCL

Keywordsi

Molecular functionHydrolase
Biological processLipid metabolism

Enzyme and pathway databases

BRENDAi3.5.1.23 2681
ReactomeiR-HSA-1660661 Sphingolipid de novo biosynthesis

Chemistry databases

SwissLipidsiSLP:000000165

Names & Taxonomyi

Protein namesi
Recommended name:
Alkaline ceramidase 1 (EC:3.5.1.23)
Short name:
AlkCDase 1
Short name:
Alkaline CDase 1
Alternative name(s):
Acylsphingosine deacylase 3
N-acylsphingosine amidohydrolase 3
Gene namesi
Name:ACER1
Synonyms:ASAH3
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 19

Organism-specific databases

EuPathDBiHostDB:ENSG00000167769.4
HGNCiHGNC:18356 ACER1
MIMi613491 gene
neXtProtiNX_Q8TDN7

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Topology

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Topological domaini1 – 27LumenalSequence analysisAdd BLAST27
Transmembranei28 – 48HelicalSequence analysisAdd BLAST21
Topological domaini49 – 57CytoplasmicSequence analysis9
Transmembranei58 – 78HelicalSequence analysisAdd BLAST21
Topological domaini79 – 81LumenalSequence analysis3
Transmembranei82 – 102HelicalSequence analysisAdd BLAST21
Topological domaini103 – 119CytoplasmicSequence analysisAdd BLAST17
Transmembranei120 – 137HelicalSequence analysisAdd BLAST18
Topological domaini138LumenalSequence analysis1
Transmembranei139 – 159HelicalSequence analysisAdd BLAST21
Topological domaini160 – 176CytoplasmicSequence analysisAdd BLAST17
Transmembranei177 – 197HelicalSequence analysisAdd BLAST21
Topological domaini198 – 206LumenalSequence analysis9
Transmembranei207 – 227HelicalSequence analysisAdd BLAST21
Topological domaini228 – 264CytoplasmicSequence analysisAdd BLAST37

Keywords - Cellular componenti

Endoplasmic reticulum, Membrane

Pathology & Biotechi

Organism-specific databases

OpenTargetsiENSG00000167769
PharmGKBiPA164714838

Chemistry databases

ChEMBLiCHEMBL3351194

Polymorphism and mutation databases

BioMutaiACER1
DMDMi74715919

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00002477451 – 264Alkaline ceramidase 1Add BLAST264

Proteomic databases

PaxDbiQ8TDN7
PeptideAtlasiQ8TDN7
PRIDEiQ8TDN7
ProteomicsDBi74314

Expressioni

Tissue specificityi

Mainly expressed in epidermis.1 Publication

Gene expression databases

BgeeiENSG00000167769
CleanExiHS_ACER1
GenevisibleiQ8TDN7 HS

Interactioni

Protein-protein interaction databases

IntActiQ8TDN7, 1 interactor
STRINGi9606.ENSP00000301452

Structurei

3D structure databases

ProteinModelPortaliQ8TDN7
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the alkaline ceramidase family.Curated

Keywords - Domaini

Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG2329 Eukaryota
ENOG4111RPN LUCA
GeneTreeiENSGT00730000110920
HOGENOMiHOG000220878
InParanoidiQ8TDN7
KOiK01441
OMAiSGYSIWM
OrthoDBiEOG091G0HT1
PhylomeDBiQ8TDN7
TreeFamiTF313019

Family and domain databases

InterProiView protein in InterPro
IPR008901 Ceramidase
PfamiView protein in Pfam
PF05875 Ceramidase, 1 hit

Sequencei

Sequence statusi: Complete.

Q8TDN7-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MPSIFAYQSS EVDWCESNFQ YSELVAEFYN TFSNIPFFIF GPLMMLLMHP
60 70 80 90 100
YAQKRSRYIY VVWVLFMIIG LFSMYFHMTL SFLGQLLDEI AILWLLGSGY
110 120 130 140 150
SIWMPRCYFP SFLGGNRSQF IRLVFITTVV STLLSFLRPT VNAYALNSIA
160 170 180 190 200
LHILYIVCQE YRKTSNKELR HLIEVSVVLW AVALTSWISD RLLCSFWQRI
210 220 230 240 250
HFFYLHSIWH VLISITFPYG MVTMALVDAN YEMPGETLKV RYWPRDSWPV
260
GLPYVEIRGD DKDC
Length:264
Mass (Da):31,095
Last modified:June 1, 2002 - v1
Checksum:iE16E5DB81D064F60
GO

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF347024 mRNA Translation: AAL83822.1
BC112122 mRNA Translation: AAI12123.1
BC112124 mRNA Translation: AAI12125.1
CCDSiCCDS12161.1
RefSeqiNP_597999.1, NM_133492.2
UniGeneiHs.352609

Genome annotation databases

EnsembliENST00000301452; ENSP00000301452; ENSG00000167769
GeneIDi125981
KEGGihsa:125981
UCSCiuc002mel.3 human

Similar proteinsi

Entry informationi

Entry nameiACER1_HUMAN
AccessioniPrimary (citable) accession number: Q8TDN7
Entry historyiIntegrated into UniProtKB/Swiss-Prot: July 25, 2006
Last sequence update: June 1, 2002
Last modified: June 20, 2018
This is version 110 of the entry and version 1 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. Human chromosome 19
    Human chromosome 19: entries, gene names and cross-references to MIM
  2. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  3. SIMILARITY comments
    Index of protein domains and families

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