Table 1 Resource information of the established iPSC line

iPSC line name

KEIOi003-A (unique name at hPSCreg)

SM9-1 (alternative name)

Institution

Keio University School of Medicine, Tokyo, Japan

Origin information

Species: human, Sex: female, Age: 70 years old, Ethnicity: Japanese

Cell source: peripheral blood mononuclear cells

Clonality

Clonal

Method of reprogramming

Electroporation using the episomal vectors: pCE-hOCT3/4, pCE-hSK, pCE-hUL, pCE-mp53DD, and pCXB-EBNA1

Name of transgenes

OCT3/4, SOX2, KLF4, L-MYC, LIN28, mutant Trp53 (removed after the establishment)

Genetic modification

Spontaneous mutation

HTRA1, c.905G>A, p.R302Q, heterozygous

Associated disease

Heterozygous HTRA1-related cerebral small vessel disease

Summary of clinical characteristics

History (age at onset):

Stroke (53 years old), depression (63 years old), normal pressure hydrocephalus (64 years old), and cervical spondylosis

Examination findings:

•No alopecia or hypertension

•Higher brain dysfunction, parkinsonism, hyperactive tendon reflexes, pathologic reflexes, and cognitive function decline

•The Japanese version of the Montreal Cognitive Assessment (MoCA-J) score: 12 points (71 years old)

•Brain MRI: Ischemic changes in the bilateral cerebral white matter, old infarcts in the basal ganglia and left thalamus, diffused microbleeds (69 years old)

Donor screening for infections

Negative for human immunodeficiency virus (HIV) antibody, hepatitis B surface (HBs) antigen, hepatitis C virus (HCV) antibody, serological tests for syphilis (STS) and treponema pallidum hemagglutination test (TPHA)