Details of Disease

General Information of Disease (ID: DISZXXG5)

Disease Name	Inclusion body myositis
Synonyms	inflammatory myopathy; Ibm; Sporadic Inclusion Body Myositis; sIBM; IBM; inclusion body myositis; sporadic inclusion body myositis
Disease Class	4A41: Idiopathic inflammatory myopathy
Definition	A slowly progressive degenerative inflammatory disorder of skeletal muscles characterized by late onset weakness of specific muscles and distinctive histopathological features.
Disease Hierarchy	DISCIXF0: Myositis disease DISZXXG5: Inclusion body myositis
ICD Code	ICD-11 ICD-11: 4A41.2 ICD-10 ICD-10: G72.8
Disease Identifiers	MONDO ID MONDO_0007827 MESH ID D018979 UMLS CUI C0238190 OMIM ID 147421 MedGen ID 68659 Orphanet ID 611 SNOMED CT ID 72315009

Drug-Interaction Atlas (DIA) of This Disease

Drug-Interaction Atlas (DIA)

This Disease is Treated as An Indication in 5 Clinical Trial Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
BYM338	DM1ARHT	Phase 3	NA	[1]
ABC008	DMTTXCF	Phase 2/3	Monoclonal antibody	[2]
Arimoclomol	DMS8QF9	Phase 2/3	Small molecular drug	[3]
Dalazatide	DMJ1NG4	Phase 1b/2a	NA	[4]
AAV1-FS344	DMAPUZY	Phase 1/2	NA	[3]
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Molecular Interaction Atlas (MIA) of This Disease

Molecular Interaction Atlas (MIA)

This Disease Is Related to 8 DTT Molecule(s)

Gene Name	DTT ID	Evidence Level	Mode of Inheritance	REF
MSTN	TTM8I2X	Limited	Biomarker	[5]
ICOSLG	TTB9Z8R	moderate	Biomarker	[6]
KLRG1	TT299E6	moderate	Biomarker	[7]
PABPC1	TTHC8EF	moderate	Biomarker	[8]
APP	TTE4KHA	Strong	Biomarker	[9]
KHDRBS1	TTAT6C7	Strong	Biomarker	[10]
PSMB10	TTPNACM	Strong	Biomarker	[11]
TARDBP	TT9RZ03	Strong	Biomarker	[10]
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⏷ Show the Full List of 8 DTT(s)

This Disease Is Related to 1 DME Molecule(s)

Gene Name	DME ID	Evidence Level	Mode of Inheritance	REF
AOC2	DE8DP90	Strong	Biomarker	[12]
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This Disease Is Related to 17 DOT Molecule(s)

Gene Name	DOT ID	Evidence Level	Mode of Inheritance	REF
FYCO1	OTKZHTZ5	Limited	Genetic Variation	[13]
CAPN3	OTCHG3YK	moderate	Altered Expression	[14]
CHMP1B	OTP715L8	moderate	Genetic Variation	[15]
DCD	OTV5PBGJ	moderate	Biomarker	[16]
H1-0	OTRLJK4Z	moderate	Biomarker	[17]
HLA-DRB3	OT5PM9N7	moderate	Genetic Variation	[18]
MAP1LC3A	OTPMGIU4	moderate	Biomarker	[19]
RRM2B	OTE8GBUR	moderate	Genetic Variation	[15]
DCTN4	OTM7C943	Strong	Biomarker	[10]
GTF2H1	OTCRXC6B	Strong	Biomarker	[10]
KDELR1	OT8YBS5G	Strong	Biomarker	[20]
NUP62	OTMN63DH	Strong	Biomarker	[10]
PLAAT4	OTI66SAJ	Strong	Altered Expression	[21]
PSME1	OTDHLJWH	Strong	Biomarker	[11]
SYNM	OTOI8TRJ	Strong	Biomarker	[22]
DNAJB6	OTMHIIAN	Definitive	Biomarker	[23]
MLF1	OTC5BKHU	Definitive	Biomarker	[23]
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⏷ Show the Full List of 17 DOT(s)

References

1	Clinical pipeline report, company report or official report of the Pharmaceutical Research and Manufacturers of America (PhRMA)
2	ClinicalTrials.gov (NCT05721573) A Phase II/III Randomized, Double-blind, Placebo-controlled, Multicenter Study to Evaluate the Efficacy and Safety of ABC008 in the Treatment of Subjects With Inclusion Body Myositis. U.S.National Institutes of Health.
3	Clinical pipeline report, company report or official report of the Pharmaceutical Research and Manufacturers of America (PhRMA)
4	Antibodies and venom peptides: new modalities for ion channels. Nat Rev Drug Discov. 2019 May;18(5):339-357.
5	Novel Therapeutic Options in Treatment of Idiopathic Inflammatory Myopathies.Curr Treat Options Neurol. 2018 Jul 23;20(9):37. doi: 10.1007/s11940-018-0521-6.
6	Upregulated inducible co-stimulator (ICOS) and ICOS-ligand in inclusion body myositis muscle: significance for CD8+ T cell cytotoxicity.Brain. 2004 May;127(Pt 5):1182-90. doi: 10.1093/brain/awh148. Epub 2004 Mar 26.
7	Highly differentiated cytotoxic T cells in inclusion body myositis.Brain. 2019 Sep 1;142(9):2590-2604. doi: 10.1093/brain/awz207.
8	Messenger RNA degradation may be inhibited in sporadic inclusion body myositis.Neurology. 2005 Aug 9;65(3):420-5. doi: 10.1212/01.wnl.0000171341.76482.15.
9	Mitochondrial dysfunction in skeletal muscle of amyloid precursor protein-overexpressing mice.J Biol Chem. 2012 Jun 8;287(24):20534-44. doi: 10.1074/jbc.M112.359588. Epub 2012 Apr 19.
10	Association between TDP-43 and mitochondria in inclusion body myositis.Lab Invest. 2019 Jul;99(7):1041-1048. doi: 10.1038/s41374-019-0233-x. Epub 2019 Feb 11.
11	Proteasomal expression, induction of immunoproteasome subunits, and local MHC class I presentation in myofibrillar myopathy and inclusion body myositis.J Neuropathol Exp Neurol. 2004 May;63(5):484-98. doi: 10.1093/jnen/63.5.484.
12	Overexpression of semicarbazide-sensitive amine oxidase in human myopathies.Muscle Nerve. 2004 Feb;29(2):261-6. doi: 10.1002/mus.10550.
13	New Developments in the Genetics of Inclusion Body Myositis.Curr Rheumatol Rep. 2018 Apr 2;20(5):26. doi: 10.1007/s11926-018-0738-0.
14	Calcium dysregulation, functional calpainopathy, and endoplasmic reticulum stress in sporadic inclusion body myositis.Acta Neuropathol Commun. 2017 Mar 22;5(1):24. doi: 10.1186/s40478-017-0427-7.
15	Mitochondrial pathology in inclusion body myositis.Neuromuscul Disord. 2015 Apr;25(4):281-8. doi: 10.1016/j.nmd.2014.12.010. Epub 2015 Jan 6.
16	Production of IL-6 by human myoblasts stimulated with Abeta: relevance in the pathogenesis of IBM.Neurology. 2001 Nov 13;57(9):1561-5. doi: 10.1212/wnl.57.9.1561.
17	Histone H1 is released from myonuclei and present in rimmed vacuoles with DNA in inclusion body myositis.Neuromuscul Disord. 2008 Jan;18(1):27-33. doi: 10.1016/j.nmd.2007.08.005. Epub 2007 Sep 20.
18	Sporadic inclusion body myositis: phenotypic variability and influence of HLA-DR3 in a cohort of 57 Australian cases.J Neurol Neurosurg Psychiatry. 2008 Sep;79(9):1056-60. doi: 10.1136/jnnp.2007.138891. Epub 2008 Feb 7.
19	Macroautophagy as a pathomechanism in sporadic inclusion body myositis.Autophagy. 2007 Jul-Aug;3(4):384-6. doi: 10.4161/auto.4245. Epub 2007 Jul 9.
20	A three-way interplay of DR4, autoantibodies and synovitis in biopsy-proven idiopathic inflammatory myositis.Rheumatol Int. 2012 Mar;32(3):611-9. doi: 10.1007/s00296-010-1637-5. Epub 2010 Dec 2.
21	RIG-I expression in perifascicular myofibers is a reliable biomarker of dermatomyositis.Arthritis Res Ther. 2017 Jul 24;19(1):174. doi: 10.1186/s13075-017-1383-0.
22	Expression of the intermediate filament protein synemin in myofibrillar myopathies and other muscle diseases.Acta Neuropathol. 2003 Jul;106(1):1-7. doi: 10.1007/s00401-003-0695-0. Epub 2003 Apr 1.
23	Non-pathogenic protein aggregates in skeletal muscle in MLF1 transgenic mice.J Neurol Sci. 2008 Jan 15;264(1-2):77-86. doi: 10.1016/j.jns.2007.07.027. Epub 2007 Sep 12.