WormBase Tree Display for Gene: WBGene00000500
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| WBGene00000500 | Evidence | Person_evidence | WBPerson434 | ||||||
|---|---|---|---|---|---|---|---|---|---|
| SMap | S_parent | Sequence | T09B4 | ||||||
| Identity | Version | 1 | |||||||
| Name | CGC_name | chn-1 | Person_evidence | WBPerson434 | |||||
| Sequence_name | T09B4.10 | ||||||||
| Molecular_name | T09B4.10 | ||||||||
| T09B4.10.1 | |||||||||
| CE29991 | |||||||||
| Other_name | 1G758 | Accession_evidence | EMBL | AF303269 | |||||
| tag-69 | CGC_data_submission | ||||||||
| CELE_T09B4.10 | Accession_evidence | NDB | BX284601 | ||||||
| Public_name | chn-1 | ||||||||
| DB_info | Database (13) | ||||||||
| Species | Caenorhabditis elegans | ||||||||
| History | Version_change | 1 | 07 Apr 2004 11:29:21 | WBPerson1971 | Event | Imported | Initial conversion from geneace | ||
| Status | Live | ||||||||
| Gene_info | Biotype | SO:0001217 | |||||||
| Gene_class | chn | ||||||||
| Allele (23) | |||||||||
| Strain | WBStrain00035717 | ||||||||
| WBStrain00003897 | |||||||||
| RNASeq_FPKM (74) | |||||||||
| GO_annotation (25) | |||||||||
| Contained_in_operon | CEOP1304 | ||||||||
| Ortholog (39) | |||||||||
| Paralog | WBGene00006781 | Caenorhabditis elegans | From_analysis | WormBase-Compara | |||||
| WBGene00015916 | Caenorhabditis elegans | From_analysis | WormBase-Compara | ||||||
| WBGene00016375 | Caenorhabditis elegans | From_analysis | WormBase-Compara | ||||||
| WBGene00019893 | Caenorhabditis elegans | From_analysis | WormBase-Compara | ||||||
| WBGene00019983 | Caenorhabditis elegans | From_analysis | WormBase-Compara | ||||||
| Structured_description | Concise_description | chn-1 encodes an ortholog of mammalian carboxyl-terminus of Hsc70interacting protein (CHIP), an E4 ubiquitin-chain elongation factor; chn-1is ubiquitously expressed; chn-1(by155) mutants are viable andsuperficially normal, but have reduced fertility and arrest as larvae ifsubjected to heat shock; chn-1 overexpression causes either embryoniclethality (if strong) or defective egg-laying and locomotion, along withconstitutive dauer formation (if weak); chn-1(by155) mutations suppressviable unc-45(e286ts) and unc-45(m94ts) mutations, but not lethalunc-45(st604) ones; chn-1(by155) mutants, unlike wild-type, show defectivesarcomeres if overexpressing unc-45 from a extrachromosomal array; CHN-1binds the ubiquitin conjugating enzyme UFD-2, which in turn binds theHsp90 cochaperone UNC-45; UNC-45 is a substrate for CHN-1- andUFD-2-dependent multiubiquitination; the parkin ortholog PDR-1 bindsCHN-1, and requires CHN-1 for self-ubiquitination; chn-1(RNAi) animalsaccumulate abnormally phosphorylated tau proteins. | Paper_evidence | WBPaper00006537 | |||||
| WBPaper00024334 | |||||||||
| WBPaper00027721 | |||||||||
| WBPaper00028312 | |||||||||
| Curator_confirmed | WBPerson567 | ||||||||
| Date_last_updated | 10 Aug 2006 00:00:00 | ||||||||
| Automated_description | Enables Hsp70 protein binding activity; ubiquitin protein ligase binding activity; and ubiquitin-ubiquitin ligase activity. Involved in several processes, including determination of adult lifespan; egg-laying behavior; and protein ubiquitination. Located in cytoplasm. Expressed in several structures, including copulatory spicule; germ line; hermaphrodite distal tip cell; intestine; and pharynx. Used to study Duchenne muscular dystrophy. Human ortholog(s) of this gene implicated in autosomal recessive spinocerebellar ataxia 16 and cerebellar ataxia type 48. Is an ortholog of human STUB1 (STIP1 homology and U-box containing protein 1). | Paper_evidence | WBPaper00065943 | ||||||
| Curator_confirmed | WBPerson324 | ||||||||
| WBPerson37462 | |||||||||
| Inferred_automatically | This description was generated automatically by a script based on data from the WS291 version of WormBase | ||||||||
| Date_last_updated | 29 Nov 2023 00:00:00 | ||||||||
| Disease_info | Experimental_model | DOID:11723 | Homo sapiens | Paper_evidence | WBPaper00031140 | ||||
| Accession_evidence | OMIM | 310200 | |||||||
| Curator_confirmed | WBPerson324 | ||||||||
| Date_last_updated | 30 Jul 2013 00:00:00 | ||||||||
| Potential_model | DOID:0080029 | Homo sapiens | Inferred_automatically | Inferred by orthology to human genes with DO annotation (HGNC:11427) | |||||
| DOID:0111746 | Homo sapiens | Inferred_automatically | Inferred by orthology to human genes with DO annotation (HGNC:11427) | ||||||
| Disease_relevance | Mutations in human Dystrophin (DMD) have been implicated in Duchenne Muscular Dystrophy and Becker muscular dystrophy that affect skeletal muscles used for movement, and heart (cardiac) muscle;; studies show that, in an C. elegans model of progressive myopathy, which consists of the elegans ortholog of human dystrophin, dys-1, combined with a mild MyoD ortholog, hlh-1, (dys-1(cx18);hlh-1(cc561ts), the muscle degeneration of affected animals is efficiently reduced by downregulation of chn-1, the homologue of the human E3/E4 ubiquitylation enzyme, CHIP; a deletion mutant of chn-1 delays the death of body-wall muscle cells and improves the motility of animals carrying mutations in dystrophin and MyoD; elimination of chn-1 function in the musculature, but not in the nervous system, is sufficient for this effect, and can be phenocopied by proteasome inhibitor treatment; these studies point to a critical role of CHIP/CHN-1-mediated ubiquitylation in the control of muscle wasting and degeneration, and identifies a potential new drug target for the treatment of the disease. | Homo sapiens | Paper_evidence | WBPaper00031140 | |||||
| Accession_evidence | OMIM | 300376 | |||||||
| 302045 | |||||||||
| 310200 | |||||||||
| 300377 | |||||||||
| Curator_confirmed | WBPerson324 | ||||||||
| Date_last_updated | 30 Jul 2013 00:00:00 | ||||||||
| Models_disease_in_annotation | WBDOannot00000220 | ||||||||
| Molecular_info | Corresponding_CDS | T09B4.10 | |||||||
| Corresponding_transcript | T09B4.10.1 | ||||||||
| Other_sequence (24) | |||||||||
| Associated_feature | WBsf649223 | ||||||||
| WBsf981027 | |||||||||
| WBsf983857 | |||||||||
| WBsf983858 | |||||||||
| WBsf1009914 | |||||||||
| WBsf1009915 | |||||||||
| WBsf219552 | |||||||||
| Experimental_info | RNAi_result (12) | ||||||||
| Expr_pattern | Expr2938 | ||||||||
| Expr3087 | |||||||||
| Expr1027014 | |||||||||
| Expr1030296 | |||||||||
| Expr1156531 | |||||||||
| Expr2009994 | |||||||||
| Expr2028235 | |||||||||
| Drives_construct | WBCnstr00008988 | ||||||||
| WBCnstr00011028 | |||||||||
| WBCnstr00011125 | |||||||||
| WBCnstr00018296 | |||||||||
| WBCnstr00037495 | |||||||||
| Construct_product | WBCnstr00008988 | ||||||||
| WBCnstr00011125 | |||||||||
| WBCnstr00018296 | |||||||||
| WBCnstr00037495 | |||||||||
| Regulate_expr_cluster | WBPaper00035567:CHN-1_interacting | ||||||||
| Antibody | WBAntibody00001121 | ||||||||
| WBAntibody00003033 | |||||||||
| WBAntibody00003037 | |||||||||
| Microarray_results (23) | |||||||||
| Expression_cluster (115) | |||||||||
| Interaction (524) | |||||||||
| Map_info | Map | I | Position | 0.876668 | Error | 0.002114 | |||
| Positive | Positive_clone | T09B4 | Inferred_automatically | From CDS info | |||||
| From sequence, transcript, pseudogene data | |||||||||
| Mapping_data | Multi_point | 4217 | |||||||
| 4841 | |||||||||
| 4805 | |||||||||
| Pseudo_map_position | |||||||||
| Reference (31) | |||||||||
| Remark | Gene name created from parsing 'genotype' field from CGC strain information | CGC_data_submission | |||||||
| Map position created from combination of previous interpolated map position (based on known location of sequence) and allele information. Therefore this is not a genetic map position based on recombination frequencies or genetic experiments. This was done on advice of the CGC. | CGC_data_submission | ||||||||
| Method | Gene |
