hydroxylysine and Glomerulonephritis

Alport's syndrome probably is a molecular disorder of basement membrane composition. Investigation of urine on basement membrane components such as hydroxylysine and its glycosides, glucosylgalactosylhydroxylysine and galactosylhydroxylysine, may be helpful for diagnosis of the disease. Urinary specimens of 33 patients and 12 siblings were investigated, and the results were compared with those of 14 healthy adults and of 29 healthy children. The urine of patients with glomerulopathies, occurring during childhood (IgA nephropathy, benign recurrent hematuria, poststreptococcal glomerulonephritis, Henoch-Schönlein nephropathy, membranoproliferative glomerulonephritis, and nephrotic syndrome due to minimal lesions), was also investigated. No marked differences between normal and diseased subjects could be demonstrated, with respect to excretion of hydroxylysine and its glycosides, in contrast to data reported in the literature.

Topics: Adolescent; Adult; Child; Child, Preschool; Female; Glomerulonephritis; Glomerulonephritis, IGA; Hematuria; Humans; Hydroxylysine; Kidney Diseases; Male; Nephritis, Hereditary; Nephrotic Syndrome

Glomerular basement membrane (GBM) were isolated from the kidneys of rats suffering from daunomycin nephrosis or nephrotoxic serum nephritis. The GBM from daunomycin nephrotic rats contained significantly less hydroxyproline, hydroxylysine and glycine than that of control rats. There was an increase in glucosamine content in the membrane. No significant change was found in the neutral sugar content. In nephrotoxic serum nephritis, the relative amounts of hydroxyproline, glycine and half-cystine were decreased, whereas the relative amounts of aspartic acid, alanine, lysine and hydroxylysine were increased. The ratio of hydroxyproline to proline and the ratio of hydroxylysine to lysine were decreased. An increase in sialic acid content and a decrease in fucose and hexosamine content and glucosyl-galactosyl-hydroxylysine content were noted in nephrotoxic nephritic GBM. These chemical structural alterations could accounted for the functional disorders of diseased GBM.

Topics: Animals; Basement Membrane; Carbohydrates; Cysteine; Daunorubicin; Glomerulonephritis; Glycine; Hydroxylysine; Hydroxyproline; Kidney Glomerulus; Nephrosis; Rats

A method was established to isolate the glomeruli from the kidney fixed in formaldehyde. O-Hydroxylysylglycosides and hydroxyproline in the isolated glomeruli from the kidney of normal subjects, diabetics and subjects with chronic glomerulonephritis were measured on the alkaline hydrolyzate of the glomeruli. O-Hydroxylysylglycosides in 1000 glomeruli were 9.42 +/- 2.68 nmoles (mean +/- S.D.) for controls, 12.92 +/- 7.36 nmoles for diabetics and 12.54 +/- 4.62 nmoles for subjects with chronic glomerulonephritis. Hydroxyproline in 1000 glomeruli from control, diabetics and subjects with chronic glomerulonephritis was 43.0 +/- 10.7, 82.0 +/- 39.3 and 52.1 +/- 21.3 nmoles, respectively. Hydroxyproline in the hyalinized glomeruli from diabetics was 123.3 +/- 22.4 nmoles/1000 glomeruli, and significantly increased in comparison with that in the glomeruli from chronic glomerulonephritis or control subjects.

Topics: Adult; Aged; Chronic Disease; Diabetes Mellitus; Glomerulonephritis; Glycoproteins; Humans; Hydroxylysine; Hydroxyproline; Kidney Glomerulus; Middle Aged

During nephrotoxic nephritis in the rat, an increased urinary excretion of glucosyl-galactosyl hydroxylysine and of galactosyl-hydroxylysine has been observed in the autologous phase of the disease. This due mainly to an elevation of the polypeptide-bound fraction of these hydroxylysyl glucosides with a molecular weight over 1,000 daltons. The levels of both urinary hydroxylysyl glucosides were correlated with proteinuria. Their increased excretion appears to originate in the lysed glomerular basement membrane. At the same stage of nephrotoxic nephritis, an increased glucosyl transferase activity could be demonstrated in the isolated glomeruli, correlated with albuminuria, attesting a higher turn-over of the disaccharide units of the glomerular basement membrane.

Topics: Albuminuria; Animals; Basement Membrane; Creatinine; Disaccharides; Diuresis; Glomerulonephritis; Hydroxylysine; Immune Sera; Kidney Cortex; Kidney Glomerulus; Male; Rabbits; Rats; Transferases

Topics: Animals; Antibodies; Autoimmune Diseases; Basement Membrane; Chromatography, Gel; Chromatography, Paper; Collagen; Disaccharides; Electrophoresis, Paper; Glomerulonephritis; Glycosides; Hydroxylysine; Immunoglobulins; Kidney Glomerulus; Male; Proteinuria; Rabbits; Rats