نتایج جستجو برای: peroxisomal disorder

تعداد نتایج: 600224  

2016
Ana Cristina Magalhães Ana Rita Ferreira Sílvia Gomes Marta Vieira Ana Gouveia Isabel Valença Markus Islinger Rute Nascimento Michael Schrader Jonathan C. Kagan Daniela Ribeiro

The human cytomegalovirus developed distinct evasion mechanisms from the cellular antiviral response involving vMIA, a virally-encoded protein that is not only able to prevent cellular apoptosis but also to inhibit signalling downstream from mitochondrial MAVS. vMIA has been shown to localize at mitochondria and to trigger their fragmentation, a phenomenon proven to be essential for the signall...

1998
Xing Xian Yu James K. Drackley Jack Odle

Peroxisomal b-oxidation and catalase activity were investigated in liver, kidney and heart from pigs at the following timepoints: within 0.5 h after birth (0 h, unfed) and at 24 h (suckled or unsuckled), 10 d (suckled or 24-h food-deprived), 21 d (suckled or 24-h food-deprived) and 5 mo (overnight food-deprived). In liver, peroxisomal b-oxidation increased about twofold at 24 h for suckled pigs...

Journal: :Human molecular genetics 2000
G Unterrainer B Molzer S Forss-Petter J Berger

Inherited defects in the X-chromosomal adrenoleukodystrophy (ALD; ABCD1) gene are the genetic cause of the severe neurodegenerative disorder X-linked adrenoleukodystrophy (X-ALD). Biochemically the accumulation of very long-chain fatty acids, caused by impaired peroxisomal beta-oxidation, is the pathognomonic characteristic of the disease. Due to the X-chromosomal inheritance of X-ALD no data a...

Journal: :Eukaryotic cell 2008
Karin Strijbis Carlo W T van Roermund Wouter F Visser Els C Mol Janny van den Burg Donna M MacCallum Frank C Odds Ekaterina Paramonova Bastiaan P Krom Ben Distel

In eukaryotes, acetyl coenzyme A (acetyl-CoA) produced during peroxisomal fatty acid beta-oxidation needs to be transported to mitochondria for further metabolism. Two parallel pathways for acetyl-CoA transport have been identified in Saccharomyces cerevisiae; one is dependent on peroxisomal citrate synthase (Cit), while the other requires peroxisomal and mitochondrial carnitine acetyltransfera...

Journal: :The Journal of Cell Biology 1992
P A Walton S J Gould R A Rachubinski S Subramani J R Feramisco

This report describes the microinjection of a purified peroxisomal protein, alcohol oxidase, from Pichia pastoris into mammalian tissue culture cells and the subsequent transport of this protein into vesicular structures. Transport was into membrane-enclosed vesicles as judged by digitonin-permeabilization experiments. The transport was time and temperature dependent. Vesicles containing alcoho...

Journal: :Molecular biology of the cell 2005
Andrew W Woodward Bonnie Bartel

Plant peroxisomal proteins catalyze key metabolic reactions. Several peroxisome biogenesis PEROXIN (PEX) genes encode proteins acting in the import of targeted proteins necessary for these processes into the peroxisomal matrix. Most peroxisomal matrix proteins bear characterized Peroxisomal Targeting Signals (PTS1 or PTS2), which are bound by the receptors PEX5 or PEX7, respectively, for import...

Journal: :The Journal of Cell Biology 1995
R K Szilard V I Titorenko M Veenhuis R A Rachubinski

Pay mutants of the yeast Yarrowia lipolytica fail to assemble functional peroxisomes. One mutant strain, pay32-1, has abnormally small peroxisomes that are often found in clusters surrounded by membraneous material. The functionally complementing gene PAY32 encodes a protein, Pay32p, of 598 amino acids (66,733 D) that is a member of the tetratricopeptide repeat family. Pay32p is intraperoxisoma...

Journal: :The Journal of biological chemistry 1990
P M Palosaari J K Hiltunen

Peroxisomal delta 3, delta 2-enoyl-CoA isomerase (EC 5.3.3.8) was studied in the liver of rats treated with clofibrate. The mitochondrial and peroxisomal isoenzymes were separated chromatographically and the peroxisomal isomerase purified to apparent homogeneity. In addition to the isomerization of 3-enoyl-CoA esters, the purified protein also catalyzed hydration of trans-2-enoyl-CoA and oxidat...

Journal: :Proceedings of the National Academy of Sciences of the United States of America 1983
M Roa G Blobel

The dramatic expansion of the peroxisomal compartment known to occur in the methanol-utilizing yeast Hansenula polymorpha on transfer from glucose- to methanol-containing media was shown to be accompanied by the synthesis of at least six major polypeptides that dominate the polypeptide pattern of total cell extracts analyzed by NaDodSO(4)/polyacrylamide gel electrophoresis. Two of these polypep...

Journal: :Endocrinology 2006
Steven Huyghe Henning Schmalbruch Karel De Gendt Guido Verhoeven Florian Guillou Paul P Van Veldhoven Myriam Baes

Inactivation of peroxisomal beta-oxidation in mice, by knocking out multifunctional protein-2 (MFP-2; also called d-bifunctional enzyme), causes male infertility. In the testis, extensive accumulations of neutral lipids were observed in Sertoli cells, beginning in prepubertal mice and evolving in complete testicular atrophy by the age of 4 months. Spermatogenesis was already severely affected a...

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