5U7Z

Human acid ceramidase (ASAH1, aCDase) self-activated

External Resource: Annotation

Domain Annotation: ECOD Classification
Protein Family Annotation
Gene Ontology: Gene Product Annotation
InterPro: Protein Family Classification
Pharos: Disease Associations

Domain Annotation: ECOD Classification ECOD Database Homepage

Chains	Family Name	Domain Identifier	Architecture	Possible Homology	Homology	Topology	Family	Provenance Source (Version)
B	CBAH	e5u7zB1	A: a+b four layers	X: Ntn/PP2C	H: Ntn	T: Penicillin acylase, catalytic domain	F: CBAH	ECOD (1.6)
D	CBAH	e5u7zD1	A: a+b four layers	X: Ntn/PP2C	H: Ntn	T: Penicillin acylase, catalytic domain	F: CBAH	ECOD (1.6)

Protein Family Annotation Pfam Database Homepage

Chains	Accession	Name	Description	Comments	Source
A, C	PF15508	beta subunit of N-acylethanolamine-hydrolyzing acid amidase (NAAA-beta)	beta subunit of N-acylethanolamine-hydrolyzing acid amidase	-	Family
B, D	PF02275	Linear amide C-N hydrolases, choloylglycine hydrolase family (CBAH)	Linear amide C-N hydrolases, choloylglycine hydrolase family	This family includes several hydrolases which cleave carbon-nitrogen bonds, other than peptide bonds, in linear amides. These include choloylglycine hydrolase (conjugated bile acid hydrolase, CBAH) EC:3.5.1.24, penicillin acylase EC:3.5.1.11 and aci ...	This family includes several hydrolases which cleave carbon-nitrogen bonds, other than peptide bonds, in linear amides. These include choloylglycine hydrolase (conjugated bile acid hydrolase, CBAH) EC:3.5.1.24, penicillin acylase EC:3.5.1.11 and acid ceramidase EC:3.5.1.23. This domain forms the alpha-subunit for members from vertebral species, see family NAAA-beta, Pfam:PF15508.	Domain

Gene Ontology: Gene Product Annotation Gene Ontology Database Homepage

Chains	Polymer	Molecular Function	Biological Process	Cellular Component
A, C	Acid ceramidase	transcription corepressor activity transcription regulator activity transcription coregulator activity protein-macromolecule adaptor activity molecular adaptor activity hydrolase activity, acting on carbon-nitrogen (but not peptide) bonds, in linear amides hydrolase activity catalytic activity hydrolase activity, acting on carbon-nitrogen (but not peptide) bonds fatty acid amide hydrolase activity N-acylsphingosine amidohydrolase activity transcription factor binding RNA polymerase II-specific DNA-binding transcription factor binding nuclear receptor binding transcription corepressor activity transcription regulator activity transcription coregulator activity protein-macromolecule adaptor activity molecular adaptor activity hydrolase activity, acting on carbon-nitrogen (but not peptide) bonds, in linear amides hydrolase activity catalytic activity hydrolase activity, acting on carbon-nitrogen (but not peptide) bonds fatty acid amide hydrolase activity N-acylsphingosine amidohydrolase activity transcription factor binding RNA polymerase II-specific DNA-binding transcription factor binding nuclear receptor binding protein binding binding DNA-binding transcription factor binding	fatty acid metabolic process oxoacid metabolic process carboxylic acid metabolic process primary metabolic process metabolic process cellular process small molecule metabolic process monocarboxylic acid metabolic process organic acid metabolic process lipid metabolic process response to peptide cellular response to tumor necrosis factor response to stimulus response to chemical fatty acid metabolic process oxoacid metabolic process carboxylic acid metabolic process primary metabolic process metabolic process cellular process small molecule metabolic process monocarboxylic acid metabolic process organic acid metabolic process lipid metabolic process response to peptide cellular response to tumor necrosis factor response to stimulus response to chemical response to cytokine cellular response to cytokine stimulus response to tumor necrosis factor regulation of biosynthetic process regulation of steroid biosynthetic process regulation of biological process regulation of cellular process regulation of lipid metabolic process steroid metabolic process regulation of steroid metabolic process biological regulation lipid biosynthetic process regulation of primary metabolic process biosynthetic process regulation of lipid biosynthetic process steroid biosynthetic process regulation of metabolic process developmental process epithelium development epidermal cell differentiation cellular developmental process epidermis development animal organ development tissue development epithelial cell differentiation skin development cell differentiation keratinocyte differentiation anatomical structure development sphingolipid biosynthetic process organic hydroxy compound metabolic process alcohol metabolic process diol biosynthetic process diol metabolic process polyol biosynthetic process membrane lipid metabolic process sphingoid biosynthetic process polyol metabolic process small molecule biosynthetic process sphingolipid metabolic process sphingosine metabolic process sphingoid metabolic process sphingosine biosynthetic process organic hydroxy compound biosynthetic process alcohol biosynthetic process membrane lipid biosynthetic process ceramide metabolic process amide biosynthetic process ceramide biosynthetic process amide metabolic process sphingolipid catabolic process lipid catabolic process catabolic process membrane lipid catabolic process ceramide catabolic process programmed necrotic cell death regulation of programmed necrotic cell death regulation of programmed cell death cell death programmed cell death	membrane-bounded organelle intracellular anatomical structure tertiary granule lumen membrane-enclosed lumen intracellular vesicle intracellular membrane-bounded organelle endomembrane system intracellular organelle lumen tertiary granule intracellular organelle cellular anatomical structure organelle lumen cytoplasmic vesicle organelle membrane-bounded organelle intracellular anatomical structure tertiary granule lumen membrane-enclosed lumen intracellular vesicle intracellular membrane-bounded organelle endomembrane system intracellular organelle lumen tertiary granule intracellular organelle cellular anatomical structure organelle lumen cytoplasmic vesicle organelle secretory granule secretory vesicle cytoplasm vesicle extracellular region lytic vacuole vacuolar lumen vacuole lysosomal lumen lysosome extracellular exosome extracellular vesicle extracellular organelle extracellular space extracellular membrane-bounded organelle ficolin-1-rich granule ficolin-1-rich granule lumen nucleus
B, D	Acid ceramidase	transcription corepressor activity transcription regulator activity transcription coregulator activity protein-macromolecule adaptor activity molecular adaptor activity hydrolase activity, acting on carbon-nitrogen (but not peptide) bonds, in linear amides hydrolase activity catalytic activity hydrolase activity, acting on carbon-nitrogen (but not peptide) bonds fatty acid amide hydrolase activity N-acylsphingosine amidohydrolase activity transcription factor binding RNA polymerase II-specific DNA-binding transcription factor binding nuclear receptor binding transcription corepressor activity transcription regulator activity transcription coregulator activity protein-macromolecule adaptor activity molecular adaptor activity hydrolase activity, acting on carbon-nitrogen (but not peptide) bonds, in linear amides hydrolase activity catalytic activity hydrolase activity, acting on carbon-nitrogen (but not peptide) bonds fatty acid amide hydrolase activity N-acylsphingosine amidohydrolase activity transcription factor binding RNA polymerase II-specific DNA-binding transcription factor binding nuclear receptor binding protein binding binding DNA-binding transcription factor binding	fatty acid metabolic process oxoacid metabolic process carboxylic acid metabolic process primary metabolic process metabolic process cellular process small molecule metabolic process monocarboxylic acid metabolic process organic acid metabolic process lipid metabolic process response to peptide cellular response to tumor necrosis factor response to stimulus response to chemical fatty acid metabolic process oxoacid metabolic process carboxylic acid metabolic process primary metabolic process metabolic process cellular process small molecule metabolic process monocarboxylic acid metabolic process organic acid metabolic process lipid metabolic process response to peptide cellular response to tumor necrosis factor response to stimulus response to chemical response to cytokine cellular response to cytokine stimulus response to tumor necrosis factor regulation of biosynthetic process regulation of steroid biosynthetic process regulation of biological process regulation of cellular process regulation of lipid metabolic process steroid metabolic process regulation of steroid metabolic process biological regulation lipid biosynthetic process regulation of primary metabolic process biosynthetic process regulation of lipid biosynthetic process steroid biosynthetic process regulation of metabolic process developmental process epithelium development epidermal cell differentiation cellular developmental process epidermis development animal organ development tissue development epithelial cell differentiation skin development cell differentiation keratinocyte differentiation anatomical structure development sphingolipid biosynthetic process organic hydroxy compound metabolic process alcohol metabolic process diol biosynthetic process diol metabolic process polyol biosynthetic process membrane lipid metabolic process sphingoid biosynthetic process polyol metabolic process small molecule biosynthetic process sphingolipid metabolic process sphingosine metabolic process sphingoid metabolic process sphingosine biosynthetic process organic hydroxy compound biosynthetic process alcohol biosynthetic process membrane lipid biosynthetic process ceramide metabolic process amide biosynthetic process ceramide biosynthetic process amide metabolic process sphingolipid catabolic process lipid catabolic process catabolic process membrane lipid catabolic process ceramide catabolic process programmed necrotic cell death regulation of programmed necrotic cell death regulation of programmed cell death cell death programmed cell death	membrane-bounded organelle intracellular anatomical structure tertiary granule lumen membrane-enclosed lumen intracellular vesicle intracellular membrane-bounded organelle endomembrane system intracellular organelle lumen tertiary granule intracellular organelle cellular anatomical structure organelle lumen cytoplasmic vesicle organelle membrane-bounded organelle intracellular anatomical structure tertiary granule lumen membrane-enclosed lumen intracellular vesicle intracellular membrane-bounded organelle endomembrane system intracellular organelle lumen tertiary granule intracellular organelle cellular anatomical structure organelle lumen cytoplasmic vesicle organelle secretory granule secretory vesicle cytoplasm vesicle extracellular region lytic vacuole vacuolar lumen vacuole lysosomal lumen lysosome extracellular exosome extracellular vesicle extracellular organelle extracellular space extracellular membrane-bounded organelle ficolin-1-rich granule ficolin-1-rich granule lumen nucleus

InterPro: Protein Family Classification InterPro Database Homepage

Chains	Accession	Name	Type
A, C	IPR029130	Acid ceramidase, N-terminal	Domain
A, C	IPR016699	Acid ceramidase-like	Family
A, C	IPR029132	Choloylglycine hydrolase/NAAA C-terminal	Domain
B, D	IPR029130	Acid ceramidase, N-terminal	Domain
B, D	IPR016699	Acid ceramidase-like	Family
B, D	IPR029132	Choloylglycine hydrolase/NAAA C-terminal	Domain

Pharos: Disease Associations Pharos Homepage Annotation

Chains	Drug Target	Associated Disease
A, C	Pharos: Q13510	Farber lipogranulomatosis spinal muscular atrophy-progressive myoclonic epilepsy syndrome Liver Cirrhosis, Experimental atrial fibrillation non-small cell lung carcinoma pilocytic astrocytoma astrocytic tumor inborn errors of metabolism lysosomal storage disease malignant mesothelioma osteosarcoma schizophrenia subependymal giant cell astrocytoma atopic eczema dermatomyositis Farber lipogranulomatosis spinal muscular atrophy-progressive myoclonic epilepsy syndrome Liver Cirrhosis, Experimental atrial fibrillation non-small cell lung carcinoma pilocytic astrocytoma astrocytic tumor inborn errors of metabolism lysosomal storage disease malignant mesothelioma osteosarcoma schizophrenia subependymal giant cell astrocytoma atopic eczema dermatomyositis glioblastoma intellectual disability lung cancer malignant colon neoplasm neurodegenerative disease ovarian cancer Sjogren syndrome epilepsy hydrops fetalis lysosomal lipid storage disorder malignant ependymoma
B, D	Pharos: Q13510	Farber lipogranulomatosis spinal muscular atrophy-progressive myoclonic epilepsy syndrome Liver Cirrhosis, Experimental atrial fibrillation non-small cell lung carcinoma pilocytic astrocytoma astrocytic tumor inborn errors of metabolism lysosomal storage disease malignant mesothelioma osteosarcoma schizophrenia subependymal giant cell astrocytoma atopic eczema dermatomyositis Farber lipogranulomatosis spinal muscular atrophy-progressive myoclonic epilepsy syndrome Liver Cirrhosis, Experimental atrial fibrillation non-small cell lung carcinoma pilocytic astrocytoma astrocytic tumor inborn errors of metabolism lysosomal storage disease malignant mesothelioma osteosarcoma schizophrenia subependymal giant cell astrocytoma atopic eczema dermatomyositis glioblastoma intellectual disability lung cancer malignant colon neoplasm neurodegenerative disease ovarian cancer Sjogren syndrome epilepsy hydrops fetalis lysosomal lipid storage disorder malignant ependymoma

RCSB PDB Core Operations are funded by the U.S. National Science Foundation (DBI-2321666), the US Department of Energy (DE-SC0019749), and the National Cancer Institute, National Institute of Allergy and Infectious Diseases, and National Institute of General Medical Sciences of the National Institutes of Health under grant R01GM157729.