Erythrocyte membrane fluidity in adult polycystic kidney disease: difference between intact cells and ghost membranes

1996 ◽  
Vol 26 (2) ◽  
pp. 171-173 ◽  
Author(s):  
K. VAREESANGTHIP ◽  
T. H. THOMAS ◽  
P. TONG ◽  
R. WILKINSON
Keyword(s):  
Kidney Disease ◽  
Membrane Fluidity ◽  
Polycystic Kidney Disease ◽  
Erythrocyte Membrane ◽  
Polycystic Kidney ◽  
Adult Polycystic Kidney Disease ◽  
Intact Cells ◽  
Erythrocyte Membrane Fluidity

scholarly journals Lack of function of an N-ethylmaleimide-sensitive thiol protein in erythrocyte membrane of autosomal dominant polycystic kidney disease.

1998 ◽  
Vol 9 (1) ◽  
pp. 1-8
Author(s):  
K Vareesangthip ◽  
R Wilkinson ◽  
T H Thomas
Keyword(s):  
Kidney Disease ◽  
Membrane Fluidity ◽  
Polycystic Kidney Disease ◽  
Erythrocyte Membrane ◽  
Cytochalasin D ◽  
Polycystic Kidney ◽  
Healthy Control ◽  
Erythrocyte Membrane Fluidity ◽  
Autosomal Dominant Polycystic Kidney ◽  
Thiol Protein

The polycystic kidney disease 1 (PKD1) gene product polycystin has been predicted to be an integral membrane protein involved in cell-cell and cell-matrix interactions. The erythrocyte membrane fluidity in autosomal dominant polycystic kidney disease (ADPKD) patients is increased, and this may be due to a membrane cytoskeletal abnormality. The abnormal erythrocyte sodium-lithium countertransport kinetics in-ADPKD are related to an altered thiol protein in the cytoskeleton. The possibility that a similar thiol protein abnormality causes the increased erythrocyte membrane fluidity in ADPKD was investigated. The membrane fluidity of intact erythrocytes from 12 ADPKD patients and 12 healthy control subjects was assessed from the fluorescence anisotropies of 1,6-diphenyl-1,3,5-hexatriene (DPH) and trimethylammonium-diphenyl-hexatriene (TMA-DPH). The effect on membrane fluidity of N-ethylmaleimide (NEM), cytochalasin D, heating at 48 degrees C for 20 min, or more specifically, liposomes containing antibodies to actin or ankyrin, was determined. In erythrocytes from healthy control subjects, the fluorescence anisotropy of DPH (mean +/- SEM: 0.223 +/- 0.001) was decreased after treatment with NEM (0.200 +/- 0.003, P < 0.001), cytochalasin D (0.206 +/- 0.006, P < 0.001), heating (0.199 +/- 0.002, P < 0.001), and antibodies to actin (0.194 +/- 0.002, P < 0.001) or ankyrin (0.196 +/- 0.002, P < 0.001). The TMA-DPH anisotropy (0.279 +/- 0.001) was also decreased after treatment with NEM (0.264 +/- 0.001, P < 0.001), cytochalasin D (0.264 +/- 0.001, P < 0.001), heating (0.265 +/- 0.001, P < 0.001), and antibodies to actin (0.262 +/- 0.002, P < 0.001) or ankyrin (0.262 +/- 0.002, P < 0.001). NEM had no additional effect on the other treatments, suggesting that its target thiol protein was associated with the cytoskeleton. In untreated erythrocytes from ADPKD patients, fluorescence anisotropies of both DPH and TMA-DPH were reduced, and none of the treatments altered the anisotropy of either DPH or TMA-DPH. In ADPKD, a cytoskeletal thiol protein is abnormal and possibly explains abnormal lipid bilayer properties and transport protein function in erythrocytes in this disease.

scholarly journals Seminal megavesicles with adult polycystic kidney disease

1998 ◽  
Vol 13 (6) ◽  
pp. 1567-1569 ◽  
Author(s):  
W. F. Hendry ◽  
D. Rickards ◽  
J. P. Pryor ◽  
L. R. Baker
Keyword(s):  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Polycystic Kidney ◽  
Adult Polycystic Kidney Disease

Tubular Function in Adult Polycystic Kidney Disease

Nephron ◽  
1979 ◽  
Vol 24 (4) ◽  
pp. 198-204 ◽  
Author(s):  
Harry Preuss ◽  
Kenneth Geoly ◽  
Michael Johnson ◽  
Alexander Chester ◽  
Alan Kliger ◽  
...  
Keyword(s):  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Tubular Function ◽  
Polycystic Kidney ◽  
Adult Polycystic Kidney Disease

scholarly journals Renal replacement therapy in adult dominant polycystic kidney disease - multicentre study

2008 ◽  
Vol 136 (Suppl. 4) ◽  
pp. 287-293
Author(s):  
Visnja Lezaic ◽  
Vladimir Ostric ◽  
Gordana Popovic ◽  
Milja Vukoje ◽  
Branislava Dragoljic ◽  
...  
Keyword(s):  
Renal Replacement Therapy ◽  
Kidney Disease ◽  
Replacement Therapy ◽  
Polycystic Kidney Disease ◽  
Causes Of Death ◽  
Family Members ◽  
Favourable Outcome ◽  
Polycystic Kidney ◽  
Adult Polycystic Kidney Disease ◽  
Av Fistula

INTRODUCTION. Adult polycystic kidney disease (APKD) is the most common hereditary kidney disease in humans. The course of the disease is accompanied by numerous complications. OBJECTIVE The aim was to assess the prevalence, clinical course and outcome of adult dominant polycystic kidney disease (ADPKD) patients on renal replacement therapy. METHOD. Medical data on 700 haemodialyzed (HD) and 500 transplanted patients treated in 10 Serbian centres from 1996 to 2000 were retrospectively analyzed. While ADPKD patients accounted for 13% of HD patients in Serbia in 2000, in the period between 1996 and 2000, the percent of patients with ADPKD in the population of patients starting HD in 8 examined centres changed from 13.5% to 6.9%. RESULTS. The total number of 180 ADPKD patients on HD was analyzed (96 males; aged 55 years at HD onset). Their HD lasted between 1 and 22 years: males started HD 1.3 years earlier and spent on HD 1.1 years less than females. In 53% of HD patients one or more family members had ADPKD but the cause of death was unknown for many family members. Hypertension was present in 75% of ADPKD patients, anaemia in 37% and other organ involvement (usually liver) was found in 53 patients. Fifty patients experienced AV fistula thrombosis and a vascular prosthesis had to be used in 9 of them. A hundred and two HD patients died (aged between 38 and 78 years, on HD for 5.3 years). The causes of death were stroke (19.6%), cardiovascular diseases (29%), infections, while 5% of patients died with the picture of acute abdomen. Among 500 transplanted patients, there were 20 patients with ADPKD (11 males, ages between 35 and 56 years at the time of transplantation) and 14 of them received graft from cadaver donor. Uni- or bilateral nephrectomy was done in 4 patients in the pretransplant preparation, and in another two early after transplantation due to urinary infection. Three patients restarted HD in the first 3 months after transplantation due to acute tubular necrosis and 10 patients died 56.5 months after the transplantation. The known causes of death were cardiovascular disease (3 patients), severe gastrointestinal bleeding (2 patients), infection (2 patients) and cancer (2 patients). CONCLUSION. The obtained results showed that it was possible to provide a favourable outcome of patients with APBB on renal replacement therapy in spite of numerous complications.

PRENATAL DIAGNOSIS OF ADULT POLYCYSTIC KIDNEY DISEASE

The Lancet ◽  
1983 ◽  
Vol 322 (8345) ◽  
pp. 337-338 ◽  
Author(s):  
Denise Main ◽  
MichaelT. Mennuti ◽  
David Cornfeld ◽  
Beverly Coleman
Keyword(s):  
Prenatal Diagnosis ◽  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Polycystic Kidney ◽  
Adult Polycystic Kidney Disease

scholarly journals Cerebral Aneurysms and Polycystic Kidney Disease: A Critical Review

1992 ◽  
Vol 19 (2) ◽  
pp. 222-227 ◽  
Author(s):  
Andres M. Lozano ◽  
Richard Leblanc
Keyword(s):  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Genetic Defect ◽  
Cerebral Aneurysms ◽  
Cooperative Study ◽  
Chromosome 16 ◽  
Polycystic Kidney ◽  
Adult Polycystic Kidney Disease ◽  
Genetically Determined ◽  
Biological Differences

ABSTRACT:The pathogenic basis of the association between adult polycystic kidney disease (APKD) and cerebral aneurysms is unknown. We have compared cerebral aneurysms in 79 patients with APKD gleaned from the literature to the sporadic aneurysm cases reported by the Cooperative Study to determine if there are significant biological differences between these two groups. Sixty-eight patients had a single aneurysm and 11 (14%) had multiple aneurysms. In APKD patients with subarachnoid hemorrhage from a single aneurysm there was a significant over-representation of males (72%, p < 0.01); and the APKD group had more aneurysms of the middle cerebral artery (37%, p < 0.05). The peak decennial incidence and mean age of rupture of APKD-associated aneurysms was younger (mean age 39.7 years, p < 0.01) and over 77% of APKD-associated aneurysms had ruptured by age 50 versus 42% for sporadic aneurysms (p < 0.001). Cerebral aneurysms co-existed with APKD in the absence of hypertension in 25% of 45 cases where the presence or absence of hypertension was recorded. These biological differences and the occurrence of aneurysms in normotensive APKD patients suggests an etiology which may be independent of hypertension and that APKD-associated aneurysms may be genetically determined. It is hypothesized that cases of inherited, familial cerebral aneurysms could be linked to a genetic defect resembling that which occurs on chromosome 16 in APKD.

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