Human induced pluripotent stem cell-derived myotubes to model inclusion body myositis

Table 1 Clinical data of the cohorts and features from IBM patients muscle’s biopsies

Sample code	Sex	Age	Time symptoms to diagnosis (months)	Rimmed Vacuoles	Inflammation	MHC1	Auto Ab	CD57	Mitochon-drial Changes	IBMFRS	Evolution
IBM1	F	83	12	+	+++	NA	All -	T clonal	YES +	8	Progressive
IBM2	M	78	12	+++	+++	+ not universal	All -	NA	NO	28	Progressive
IBM3	M	63	36	+	+++	Universal	All -	NA	YES +	27	Progressive
CTL1	M	69
CTL2	F	52
CTL3	M	40
CTL4	F	61

Abbreviations: IBM: Inclusion body myositis patients; CTL: healthy control subjects; Sex: F:female; M:male; Rimmed vacuoles: from 0 to +++; Inflammation: from 0 to +++; MHC1: Major Histocompatibility Complex: Universal; NA: not available; + not universal: splattered pattern; Auto Ab: Autoantibodies: Myositis specific and associated auto antibodies include: Anti-Jo-1, anti-SRP, anti-Mi2, anti-TIF1gamma, anti-MDA5, anti-SAE, anti-NXP2 and anti-HMGCR (specific) and anti-PM/Scl, anti-Ro, anti-La, and anti-U1RNP (associated); Mitochondrial changes: Yes or No. Yes: RRF and/or COX neg. Yes + (strong alterations). NO: no changes; Age and gender-paired distribution between cohorts (p = NS). IBMFRS: Inclusion Body Myositis Functional Rating Scale (from 0 -inability- to 40 points -health-); Evolution: Progressive vs. stable phase of the disease

ISSN: 2051-5960