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Published on: June 30, 2023
Liver mitochondrial function in familial amyloidotic polyneuropathy
O Castro e Silva1, A K Sankarankutty, M E J Souza
1Special Liver Transplantation Unit, Departments of Surgery and Anatomy, Faculty of Medicine of Ribeirào Preto, University of São Paulo, São Paulo, Brazil. orlando@fmrp.usp.br
Transplantation Proceedings
|May 6, 2008
Summary
Familial amyloidotic polyneuropathy (FAP) patients undergoing liver transplantation show impaired hepatic mitochondrial function. This study reveals a decreased liver energy status in FAP patients compared to controls.
Area of Science:
- Hepatology
- Mitochondrial Biology
- Transplantation Medicine
Background:
- Familial amyloidotic polyneuropathy (FAP) is a rare genetic disorder.
- Liver transplantation is a potential treatment for FAP.
- Hepatic mitochondrial dysfunction may contribute to FAP progression.
Purpose of the Study:
- To evaluate hepatic mitochondrial function in FAP patients before liver transplantation.
- To compare mitochondrial function between FAP patients and a control group.
Main Methods:
- Biopsies of liver tissue were obtained from FAP patients (n=8) and controls (n=15).
- Mitochondrial respiration (oxygen consumption) was measured using polarography.
- Mitochondrial membrane potential was assessed spectrofluorometrically.
Main Results:
- FAP patients exhibited significantly lower State 3 respiration (28 vs 47 natoms O/min/mg protein, P < .05).
- The respiratory control ratio was significantly reduced in FAP patients (1.7 vs 3.6, P < .05).
- Mitochondrial membrane potential was similar between groups (135 mV).
Conclusions:
- FAP is associated with impaired hepatic mitochondrial energy metabolism.
- Reduced mitochondrial respiration suggests a compromised energy status in FAP livers.
- These findings highlight potential targets for therapeutic interventions in FAP.
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