Metachromatic leukodystrophy. Report of siblings with the juvenile type of metachromatic leukodystrophy.

Two sisters with juvenile metachromatic leukodystrophy are described. The patients were 17 and 20 years old. The younger sister died and an autopsy was performed.

Effect of natural peptide YY on blood flow and exocrine secretion of pancreas in dogs.

Little is known regarding the mechanism by which peptide YY exerts an inhibitory effect on exocrine pancreatic secretion. The purpose of this study is to determine if peptide YY affects pancreatic blood flow with simultaneous measurement of exocrine pancreatic secretion in dogs. Pancreatic blood flow was measured by a laser Doppler flowmeter which allows continuous measurement of tissue blood flow.

Role of sulphated tyrosine residue in influencing the biologic activity of human cholecystokinin-33.

To evaluate the role of the sulphated tyrosine residue in position 27 in human cholecystokinin-33, parallel bioassay of the sulphated form of human cholecystokinin-33 and the unsulphated form of human cholecystokinin-33 was performed on the pancreatic protein secretion. Both peptides increased the protein output in a dose-related manner. However, the sulphated form possessed a considerably higher activity than the sulphated form.

Skin graft including subcutaneous vein: experimental study and clinical applications.

Skin grafting, using full and split thickness techniques, may yield poor results when satisfactory circulation is not restored in the recipient bed. Using a rabbit ear model with a vein located in the center of the graft, donor skin and subcutaneous vein were raised simultaneously, and the vein was preserved, resulting in a viable passage for inflow and outflow. Grafts were shown to survive in this experimental group when circulation was satisfactory after venous preservation.

Studies on peptides CLVIII. Model experiments for the synthesis of open-chain unsymmetrical cystine-peptides.

Treatment of a mixture of Cys(R)(O) and Cys(R') with an acid was found to generate cystine in fairly good yields, when suitable R, R', and an acid were selected. An unsymmetrical cystine peptide was prepared by treatment of a mixture of Z(OMe)-Cys(R) (0)-Ala-NH2 (R = Acm or MBzl) and Z(OMe)-Cys(MBzl)-Gly-OBzl with TFA or 1 M TFMSA/TFA3. Oxytocin was obtained in an excellent yield by TFA treatment of the protected peptide containing Cys(Acm)(0) and Cys(MBzl).

Assessment of neuromedin B polyclonal antibodies as molecular probes in neural tissue.

Five unique, high affinity rabbit polyclonal antibodies against neuromedin B were characterized in a radioimmunoassay in terms of the following parameters: pH and type of buffer, ionic strength, and non-ionic detergents in order to optimize immunoglobulin-peptide interaction; specificity using peptides of the bombesin family, in addition to the tachykinin substance P; and affinity to neuromedin B. Optimum conditions included acidic pH (5.25), high ionic strength (greater than 0.

Effect of synthetic human cholecystokinin-33 on exocrine pancreas.

Synthetic human cholecystokinin-33 was first evaluated with respect to the biological activity on the pancreatic protein secretion. Human cholecystokinin-33 increased pancreatic protein secretion in a dose-related manner. The relative molar potency of this substance compared to that of synthetic cholecystokinin-8 (taken as 1.

Effects of human pancreatic polypeptide on exocrine pancreatic function in conscious rats.

The effect of newly synthesized human pancreatic polypeptide (hPP) on pancreatic secretion stimulated by a diversion of bile-pancreatic juice (BPJ) from the intestine, and plasma hPP concentration were examined in conscious rats. Exogenous hPP infusion (1, 10 micrograms/kg/hr) inhibited pancreatic protein, bicarbonate and fluid outputs during BPJ diversion in a dose-dependent manner. During 1 and 10 micrograms/kg/hr hPP infusion plasma hPP immunoreactivity increased to 369 +/- 22.

Structure-activity relationship of hylambatin and its fragments as studied in the guinea-pig ileum.

Hylambatin (Hyl), a dodecapeptide isolated from the skin of the African frog, Hylambates maculatus, belongs to the family of tachykinin or physalaemin-like peptides. Hylambatin and its 12 fragments were tested in the guinea-pig ileum preparation for contractile activities. All fragments except 3 had contractile activities.

Progression of the phenotype of transformed cells after growth stimulation of cells by a human papillomavirus type 16 gene function.

Alteration of the growth properties of the established murine fibroblast cell lines NIH 3T3 and 3Y1 was studied in monolayer cultures and in cells suspended in semisolid medium after introduction of a cloned human papillomavirus type 16 (HPV16) DNA. HPV 16 DNA stimulated both cell lines to grow beyond their saturation densities in monolayer cultures without any apparent morphological changes or tendency to pile up. These cells were also stimulated to grow in soft agar.

Effect of synthetic neuromedin U-8 and U-25, novel peptides identified in porcine spinal cord, on splanchnic circulation in dogs.

Two novel peptides which exert a potent stimulant effect on rat uterus smooth muscle have recently been identified in porcine spinal cord. These peptides designated neuromedin U-8 and U-25 have been reported to exert a hypertensive effect in rats. But further biological activities are not known.

Neurotensin receptors on the rat liver plasma membranes.

Neurotensin (NT) is now classified as a brain-gut peptide in the central nervous system and gastrointestinal tract. In the present study, we characterized the NT receptors on the rat liver plasma membranes. The specific binding of [3H]NT was time dependent, reversible, and saturable.

Fast atom bombardment/tandem mass spectrometry of physalaemin-like peptides.

Fragment ion spectra obtained from collision-induced decomposition of protonated molecular ions have been used to determine amino acid sequences of several physalaemin-like peptides which were recently purified from rabbit stomach. This technique was chosen because the peptides were available in microgram quantities and were anticipated to contain pyroglutamate as the blocked N-terminal residue. Such spectra of several synthetic analogs of the naturally occurring peptides were obtained and analyzed to confirm the veracity of this peptide sequencing strategy.

Proteolysis of human C-reactive protein produces peptides with potent immunomodulating activity.

We have studied the ability of human C-reactive protein to modulate the immune response in vitro. Whereas native C-reactive protein did not induce phagocytic leukocytes to chemotax or to produce superoxide, treatment of purified C-reactive protein with human neutrophil-derived acid proteases produced substances with potent effects on leukocyte function. Close examination of the primary structure of human C-reactive protein revealed three regions evenly distributed throughout the protein each of which contain peptide sequences closely resembling the amino acid sequence of the immunomodulator peptide tuftsin, Thr-Lys-Pro-Arg.