Loss of gastric interstitial cells of Cajal in patients with hereditary transthyretin amyloidosis

Background: Hereditary transthyretin (TTR) amyloidosis is a systemic neuropathic disorder caused by TTR gene mutations. Gastrointestinal complications are common and the underlying mechanisms remain unclear. The interstitial cells of Cajal (ICC) function as pacemaker cells in the gastrointestinal tract and are important for gastrointestinal motility. The aim of this study was to investigate the densities of gastric ICC and nerves in patients with TTR amyloidosis compared to non-amyloidosis controls.Methods: Antral wall autopsy specimens from 11 Japanese ATTR V30M patients and 10 controls were analyzed with immunohistochemistry and computerized analysis. Antibodies to c-Kit and TMEM16A were used to assess ICC and an antibody to PGP 9.5 was used to assess nervous tissue. The study was approved by a Japanese ethical committee.Results: The densities of c-Kit-immunoreactive (IR) ICC were significantly lower in the circular and longitudinal muscle layers of patients compared to controls (p = 0.004 for both). Equivalent results were found for TMEM 16A-IR ICC. There were no significant differences in PGP 9.5-IR cells in the circular or longitudinal muscle layers between patients and controls (p = 0.173 and 0.099, respectively).Conclusions: A loss of gastrointestinal ICC may be an important factor for the digestive disturbances in hereditary TTR amyloidosis.

MEDICIN OCH HÄLSOVETENSKAP Medicinsk bioteknologi Medicinsk bioteknologi hsv//swe
MEDICAL AND HEALTH SCIENCES Medical Biotechnology Medical Biotechnology hsv//eng
Amyloidosis
enteric nervous system
functional
gastrointestinal disorders
hereditary
interstitial cell of Cajal
transthyretin

Obayashi, KonenDepartment of Diagnostic Medicine, Graduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan (författare)
Ando, YukioDepartment of Diagnostic Medicine, Graduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan (författare)
Karling, PontusUmeå universitet,Medicin(Swepub:umu)peakag84 (författare)
Anan, IntissarUmeå universitet,Medicin(Swepub:umu)inrann97 (författare)
Umeå universitetMedicin (creator_code:org_t)

Av författaren/redakt...: Wixner, Jonas; Obayashi, Konen; Ando, Yukio; Karling, Pontus; Anan, Intissar

Om ämnet

MEDICIN OCH HÄLSOVETENSKAP: MEDICIN OCH HÄLS ...; och Medicinsk biotek ...; och Medicinsk biotek ...

Kungliga biblioteket hanterar dina personuppgifter i enlighet med EU:s dataskyddsförordning (2018), GDPR. Läs mer om hur det funkar här.
Så här hanterar KB dina uppgifter vid användning av denna tjänst.