| IED ID | IndEnz0002015336 |
| Enzyme Type ID | protease015336 |
| Protein Name |
Indian hedgehog protein IHH HHG-2 Cleaved into: Indian hedgehog protein N-product; Indian hedgehog protein C-product |
| Gene Name | IHH |
| Organism | Homo sapiens (Human) |
| Taxonomic Lineage | cellular organisms Eukaryota Opisthokonta Metazoa Eumetazoa Bilateria Deuterostomia Chordata Craniata Vertebrata Gnathostomata (jawed vertebrates) Teleostomi Euteleostomi Sarcopterygii Dipnotetrapodomorpha Tetrapoda Amniota Mammalia Theria Eutheria Boreoeutheria Euarchontoglires Primates Haplorrhini Simiiformes Catarrhini Hominoidea (apes) Hominidae (great apes) Homininae Homo Homo sapiens (Human) |
| Enzyme Sequence | MSPARLRPRLHFCLVLLLLLVVPAAWGCGPGRVVGSRRRPPRKLVPLAYKQFSPNVPEKTLGASGRYEGKIARSSERFKELTPNYNPDIIFKDEENTGADRLMTQRCKDRLNSLAISVMNQWPGVKLRVTEGWDEDGHHSEESLHYEGRAVDITTSDRDRNKYGLLARLAVEAGFDWVYYESKAHVHCSVKSEHSAAAKTGGCFPAGAQVRLESGARVALSAVRPGDRVLAMGEDGSPTFSDVLIFLDREPHRLRAFQVIETQDPPRRLALTPAHLLFTADNHTEPAARFRATFASHVQPGQYVLVAGVPGLQPARVAAVSTHVALGAYAPLTKHGTLVVEDVVASCFAAVADHHLAQLAFWPLRLFHSLAWGSWTPGEGVHWYPQLLYRLGRLLLEEGSFHPLGMSGAGS |
| Enzyme Length | 411 |
| Uniprot Accession Number | Q14623 |
| Absorption | |
| Active Site | |
| Activity Regulation | |
| Binding Site | |
| Calcium Binding | |
| catalytic Activity | |
| DNA Binding | |
| EC Number | |
| Enzyme Function | FUNCTION: Intercellular signal essential for a variety of patterning events during development. Binds to the patched (PTC) receptor, which functions in association with smoothened (SMO), to activate the transcription of target genes. Implicated in endochondral ossification: may regulate the balance between growth and ossification of the developing bones. Induces the expression of parathyroid hormone-related protein (PTHRP) (By similarity). {ECO:0000250, ECO:0000269|PubMed:21537345}. |
| Temperature Dependency | |
| PH Dependency | |
| Pathway | |
| nucleotide Binding | |
| Features | Beta strand (9); Chain (3); Glycosylation (1); Helix (6); Lipidation (2); Metal binding (12); Mutagenesis (1); Natural variant (6); Sequence conflict (3); Signal peptide (1); Site (4); Turn (1) |
| Keywords | 3D-structure;Autocatalytic cleavage;Calcium;Cell membrane;Developmental protein;Disease variant;Glycoprotein;Hydrolase;Lipoprotein;Membrane;Metal-binding;Palmitate;Protease;Reference proteome;Secreted;Signal;Zinc |
| Interact With | Q9BWV1 |
| Induction | |
| Subcellular Location | SUBCELLULAR LOCATION: [Indian hedgehog protein N-product]: Cell membrane {ECO:0000250}; Lipid-anchor {ECO:0000250}; Extracellular side {ECO:0000250}. Note=The N-terminal peptide remains associated with the cell surface. {ECO:0000250}.; SUBCELLULAR LOCATION: [Indian hedgehog protein C-product]: Secreted, extracellular space {ECO:0000250}. Note=The C-terminal peptide diffuses from the cell. {ECO:0000250}.; SUBCELLULAR LOCATION: Cell membrane {ECO:0000269|PubMed:21537345}. |
| Modified Residue | |
| Post Translational Modification | PTM: The C-terminal domain displays an autoproteolysis activity and a cholesterol transferase activity. Both activities result in the cleavage of the full-length protein and covalent attachment of a cholesterol moiety to the C-terminal of the newly generated N-terminal fragment (N-product). The N-product is the active species in both local and long-range signaling, whereas the C-product has no signaling activity (By similarity). {ECO:0000250}.; PTM: Cholesterylation is required for N-product targeting to lipid rafts and multimerization. {ECO:0000269|PubMed:21537345}.; PTM: Palmitoylated. N-palmitoylation is required for N-product multimerization and full activity. {ECO:0000269|PubMed:21537345, ECO:0000269|PubMed:9593755}. |
| Signal Peptide | SIGNAL 1..27; /evidence=ECO:0000305 |
| Structure 3D | X-ray crystallography (8) |
| Cross Reference PDB | 3K7G; 3K7H; 3K7I; 3K7J; 3N1F; 3N1M; 3N1O; 3N1P; |
| Mapped Pubmed ID | 10050855; 10619433; 10966113; 10984056; 11017805; 11027307; 11476578; 11486055; 11841368; 12015973; 12123571; 12368206; 12372301; 12525541; 12569124; 12917489; 14520411; 14651602; 14770182; 15019948; 15075292; 15105288; 15107406; 15110707; 15146555; 15163793; 15292211; 15294024; 15300262; 15356051; 15618519; 15647319; 15963974; 16136078; 16282375; 16322088; 16390857; 16611729; 16630821; 16647304; 16871364; 16980583; 17086203; 17126548; 17210683; 17360475; 17387386; 17486609; 17504940; 17504941; 17507410; 17609110; 17641202; 17960137; 17962696; 17967162; 18264092; 18391951; 18391952; 18477453; 18497258; 18502753; 18534984; 18559595; 18629882; 18711132; 18788453; 18794898; 18829534; 19002207; 19074837; 19081070; 19176481; 19196978; 19213939; 19218434; 19346256; 19365551; 19445942; 19453261; 19464397; 19561609; 19561611; 19603532; 19856079; 19948480; 20007344; 20189936; 20400458; 20412775; 20546612; 20585641; 20683927; 20724312; 20839884; 20844013; 20844016; 20846217; 20854074; 20881264; 21167467; 21257310; 21339334; 21357747; 21640343; 21642379; 21659505; 21664575; 21664576; 21664577; 21695114; 21802063; 21816240; 21842183; 21988832; 22123293; 22234151; 22277298; 22326621; 22406540; 22467855; 22469853; 22486854; 22507129; 22609552; 22677548; 22750747; 22817660; 22902404; 22981989; 22986504; 23026747; 23112049; 23118222; 23303278; 23403897; 23418531; 23575478; 23719536; 23725801; 23786654; 23867461; 23867465; 23883825; 23900540; 24302888; 24344235; 24582806; 24784881; 24786088; 24844414; 24925320; 24991722; 25519748; 25692887; 25888497; 25958162; 26201092; 26649570; 26691363; 26705100; 26963384; 27694322; 27801732; 28205343; 28574612; 29088246; 29155992; 29748156; 30926792; 31087761; 31820036; 31847127; 32209048; 32235006; 32311039; 34070546; 34530144; 7885476; 7985023; 8689684; 8824192; 8898207; 8906787; 8906794; |
| Motif | |
| Gene Encoded By | |
| Mass | 45,251 |
| Kinetics | |
| Metal Binding | METAL 94; /note="Calcium 1"; /evidence="ECO:0000269|PubMed:20519495"; METAL 95; /note="Calcium 1"; /evidence="ECO:0000269|PubMed:20519495"; METAL 95; /note="Calcium 2"; /evidence="ECO:0000269|PubMed:20519495"; METAL 100; /note="Calcium 1"; /evidence="ECO:0000269|PubMed:20519495"; METAL 130; /note="Calcium 1; via carbonyl oxygen"; /evidence="ECO:0000269|PubMed:20519495"; METAL 131; /note="Calcium 1"; /evidence="ECO:0000269|PubMed:20519495"; METAL 131; /note="Calcium 2"; /evidence="ECO:0000269|PubMed:20519495"; METAL 134; /note="Calcium 2"; /evidence="ECO:0000269|PubMed:20519495"; METAL 136; /note="Calcium 2"; /evidence="ECO:0000269|PubMed:20519495"; METAL 145; /note="Zinc"; /evidence="ECO:0000269|PubMed:20519495, ECO:0000269|PubMed:21537345"; METAL 152; /note="Zinc"; /evidence="ECO:0000269|PubMed:20519495, ECO:0000269|PubMed:21537345"; METAL 187; /note="Zinc"; /evidence="ECO:0000269|PubMed:20519495, ECO:0000269|PubMed:21537345" |
| Rhea ID | |
| Cross Reference Brenda |