Annotations: 3GKH

Domain Annotation: SCOP2 Classification SCOP2 Database Homepage

Chains	Type	Family Name	Domain Identifier	Family Identifier	Provenance Source (Version)
A	SCOP2B Superfamily	Frizzled domain-like	8090917	3001950	SCOP2B (2022-06-29)

Domain Annotation: ECOD Classification ECOD Database Homepage

Chains	Family Name	Domain Identifier	Architecture	Possible Homology	Homology	Topology	Family	Provenance Source (Version)
A	NPC1_N	e3gkhA1	A: few secondary structure elements	X: Frizzled cysteine-rich domain-related (From Topology)	H: Frizzled cysteine-rich domain-related (From Topology)	T: Frizzled cysteine-rich domain-related	F: NPC1_N	ECOD (1.6)

Protein Family Annotation Pfam Database Homepage

Chains	Accession	Name	Description	Comments	Source
A	PF16414	Niemann-Pick C1 N terminus (NPC1_N)	Niemann-Pick C1 N terminus	This is the N-terminal domain of Niemann-Pick C1 family proteins. This family of proteins mediates transport of cholesterol from the intestinal lumen to enterocytes. This domain contains a cholesterol-binding pocket [1-2].	Domain

Gene Ontology: Gene Product Annotation Gene Ontology Database Homepage

Chains	Polymer	Molecular Function	Biological Process	Cellular Component
A	Niemann-Pick C1 protein	molecular transducer activity transmembrane signaling receptor activity signaling receptor activity protein binding binding virus receptor activity exogenous protein binding sterol binding organic cyclic compound binding alcohol binding steroid binding lipid binding cholesterol binding small molecule binding molecular transducer activity transmembrane signaling receptor activity signaling receptor activity protein binding binding virus receptor activity exogenous protein binding sterol binding organic cyclic compound binding alcohol binding steroid binding lipid binding cholesterol binding small molecule binding lipid transporter activity transporter activity	response to stimulus response to chemical response to xenobiotic stimulus cellular localization intracellular lipid transport transport lipid localization lipid transport intracellular transport establishment of localization localization macromolecule localization establishment of localization in cell cellular process response to stimulus response to chemical response to xenobiotic stimulus cellular localization intracellular lipid transport transport lipid localization lipid transport intracellular transport establishment of localization localization macromolecule localization establishment of localization in cell cellular process biological process involved in interspecies interaction between organisms symbiont entry into host cell viral process symbiont entry into host viral life cycle biological process involved in interaction with host biological process involved in symbiotic interaction cellular catabolic process catabolic process cellular metabolic process autophagy metabolic process process utilizing autophagic mechanism cholesterol transport sterol transport organic hydroxy compound transport cell death programmed cell death cellular response to stimulus response to lipid response to endogenous stimulus cellular response to organic cyclic compound cellular response to endogenous stimulus cellular response to chemical stimulus response to hormone response to steroid hormone cellular response to lipid cellular response to steroid hormone stimulus response to organic cyclic compound cellular response to hormone stimulus intracellular cholesterol transport intracellular sterol transport intestinal lipid absorption multicellular organismal process digestion system process intestinal absorption digestive system process intestinal cholesterol absorption lipid digestion cellular response to low-density lipoprotein particle stimulus response to lipoprotein particle response to nitrogen compound cellular response to lipoprotein particle stimulus cholesterol efflux carbohydrate metabolic process glucan metabolic process polysaccharide metabolic process oligosaccharide metabolic process primary metabolic process macromolecule metabolic process cyclodextrin metabolic process adult walking behavior adult locomotory behavior behavior adult behavior locomotory behavior walking behavior regulation of biological process regulation of cellular process regulation of epithelial cell apoptotic process negative regulation of programmed cell death regulation of apoptotic process biological regulation negative regulation of biological process regulation of programmed cell death epithelial cell apoptotic process negative regulation of apoptotic process negative regulation of epithelial cell apoptotic process negative regulation of cellular process apoptotic process developmental process multicellular organism development hepaticobiliary system development gland development animal organ development anatomical structure development liver development system development neurogenesis cellular developmental process cell differentiation nervous system development oxoacid metabolic process steroid metabolic process organic hydroxy compound metabolic process carboxylic acid metabolic process organic acid metabolic process bile acid metabolic process small molecule metabolic process monocarboxylic acid metabolic process lipid metabolic process membrane organization cellular component organization membrane raft organization cellular component organization or biogenesis import into cell endocytosis vesicle-mediated transport negative regulation of cellular catabolic process macroautophagy regulation of autophagy negative regulation of cellular metabolic process negative regulation of catabolic process negative regulation of metabolic process regulation of cellular metabolic process regulation of catabolic process negative regulation of macroautophagy regulation of macroautophagy negative regulation of autophagy regulation of cellular catabolic process regulation of metabolic process sterol homeostasis chemical homeostasis homeostatic process cholesterol homeostasis lipid homeostasis secondary alcohol metabolic process sterol metabolic process cholesterol metabolic process alcohol metabolic process carbohydrate derivative biosynthetic process cellular biosynthetic process glycoprotein biosynthetic process carbohydrate derivative metabolic process glycoprotein metabolic process protein glycosylation macromolecule biosynthetic process biosynthetic process glycosylation macromolecule modification macromolecule glycosylation protein metabolic process protein modification process cholesterol storage lipid storage maintenance of location gene expression vacuolar transport lysosomal transport signaling negative regulation of signaling negative regulation of intracellular signal transduction regulation of signal transduction intracellular signal transduction negative regulation of cell communication cell communication negative regulation of TORC1 signaling regulation of intracellular signal transduction negative regulation of TOR signaling signal transduction regulation of TORC1 signaling TOR signaling regulation of signaling regulation of TOR signaling negative regulation of response to stimulus negative regulation of signal transduction regulation of response to stimulus regulation of cell communication TORC1 signaling protein localization establishment of protein localization establishment of protein localization to membrane localization within membrane protein localization to membrane cellular macromolecule localization response to metal ion response to cadmium ion	membrane cellular anatomical entity membrane-bounded organelle intracellular organelle organelle envelope intracellular anatomical structure organelle nuclear envelope nucleus intracellular membrane-bounded organelle endomembrane system extracellular exosome extracellular vesicle extracellular organelle membrane cellular anatomical entity membrane-bounded organelle intracellular organelle organelle envelope intracellular anatomical structure organelle nuclear envelope nucleus intracellular membrane-bounded organelle endomembrane system extracellular exosome extracellular vesicle extracellular organelle extracellular region extracellular space vesicle extracellular membrane-bounded organelle endosome membrane endosome intracellular vesicle late endosome membrane cytoplasmic vesicle membrane bounding membrane of organelle cytoplasmic vesicle vesicle membrane late endosome cytoplasm organelle membrane vacuolar membrane lytic vacuole vacuole lytic vacuole membrane lysosomal membrane lysosome Golgi apparatus perinuclear region of cytoplasm cell periphery plasma membrane membrane microdomain membrane raft endoplasmic reticulum

InterPro: Protein Family Classification InterPro Database Homepage

Chains	Accession	Name	Type
A	IPR032190	Niemann-Pick C1, N-terminal	Domain
A	IPR004765	NPC1-like	Family
A	IPR000731	Sterol-sensing domain	Domain
A	IPR053958	HMGCR/SNAP/NPC1-like, sterol-sensing domain	Domain
A	IPR003392	Protein patched/dispatched	Family
A	IPR053956	NPC1, middle luminal domain	Domain

Pharos: Disease Associations Pharos Homepage Annotation

Chains	Drug Target	Associated Disease
A	Pharos: O15118	Niemann-Pick disease ophthalmoplegia Niemann-Pick disease, type C1 obesity disorder gastrointestinal stromal tumor Ataxia atherosclerosis cirrhosis of liver liver disease brucellosis osteosarcoma sphingolipidosis histiocytosis inborn errors of metabolism large cell medulloblastoma Niemann-Pick disease ophthalmoplegia Niemann-Pick disease, type C1 obesity disorder gastrointestinal stromal tumor Ataxia atherosclerosis cirrhosis of liver liver disease brucellosis osteosarcoma sphingolipidosis histiocytosis inborn errors of metabolism large cell medulloblastoma lysosomal storage disease metabolic syndrome X ovarian cancer Gaucher disease hydrops fetalis adrenal cortex carcinoma intellectual disability leukodystrophy lung cancer malignant pancreatic neoplasm C syndrome non-Langerhans cell histiocytosis psoriasis heart disease tuberculosis arteriosclerosis disorder lung carcinoma medulloblastoma cholestasis Niemann-Pick disease type C Down syndrome

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.