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Muscular dystrophy-related quantitative and chemical changes in adenohypophysis GH-cells in golden retrievers.

de Lima, AR, Nyengaard, JR, Jorge, AA, Balieiro, JC, Peixoto, C, Fioretto, ET, Ambrósio, CE, Miglino, MA, Zatz, M and Ribeiro, AA (2007) Muscular dystrophy-related quantitative and chemical changes in adenohypophysis GH-cells in golden retrievers. Growth Horm IGF Res, 17 (6). pp. 480-491.

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Abstract

Duchenne muscular dystrophy (DMD) is a recessive X-linked lethal condition which affects a boy in every 3300 births. It is caused by the absence of dystrophin, a protein occurring especially within the musculoskeletal system and in neurons in specific regions of the central nervous system (CNS). Growth hormone (GH) inhibition is believed to decrease the severity of DMD and could perhaps be used in its treatment. However, the underlying pathological mechanism is not known. The golden retriever muscular dystrophy dog (GRMD) represents an animal model in the study of DMD. In this paper we investigated the morphological aspects of the adenohypophysis as well as the total number and size of GH-granulated cells using design-based stereological methods in a limited number of dystrophic and healthy golden retrievers. GH-cells were larger (32.4%) in dystrophic dogs than in healthy animals (p=0.01) and they occupied a larger portion (62.5%) of the adenohypophysis volume (p=0.01) without changes in either adenohypophysis volume (p=0.893) or total number of GH-granulated cells (p=0.869). With regard to ultrastructure, granulated cells possessed double-layer electron-dense granules which were evenly distributed in the cytosol. Furthermore, these granules in dystrophic animals occupied a larger proportion of GH-granulated cell volume (66.9%; p=0.008) as well as of all GH-cells in the whole pars distalis of adenohypophysis (77.3%; p=0.035), albeit IGF-1 serum concentration was lower in severe cases. This suggests difficulties in the GH secretion that might possibly be associated to dystrophin absence. In contrast to earlier reports, our data suggest that a lower IGF-1 concentration may be more related to a severe, as opposed to a benign, clinical form of muscular dystrophy.

Item Type: Article
Authors :
NameEmailORCID
de Lima, ARUNSPECIFIEDUNSPECIFIED
Nyengaard, JRUNSPECIFIEDUNSPECIFIED
Jorge, AAUNSPECIFIEDUNSPECIFIED
Balieiro, JCUNSPECIFIEDUNSPECIFIED
Peixoto, CUNSPECIFIEDUNSPECIFIED
Fioretto, ETUNSPECIFIEDUNSPECIFIED
Ambrósio, CEUNSPECIFIEDUNSPECIFIED
Miglino, MAUNSPECIFIEDUNSPECIFIED
Zatz, MUNSPECIFIEDUNSPECIFIED
Ribeiro, AAUNSPECIFIEDUNSPECIFIED
Date : December 2007
Identification Number : https://doi.org/10.1016/j.ghir.2007.06.001
Uncontrolled Keywords : Animals, Body Weight, Cell Count, Cell Size, Creatine Kinase, Cytoplasmic Granules, Disease Progression, Dogs, Dystrophin, Genotype, Growth Hormone, Insulin-Like Growth Factor I, Male, Muscular Dystrophy, Animal, Pituitary Gland, Anterior, Polymerase Chain Reaction, Reference Values
Related URLs :
Depositing User : Symplectic Elements
Date Deposited : 17 May 2017 10:14
Last Modified : 17 May 2017 10:14
URI: http://epubs.surrey.ac.uk/id/eprint/826976

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