PMID: 3212889Nov 1, 1988Paper

Otic pathology of caprine beta-mannosidosis

Veterinary Pathology
J A RenderM Z Jones

Abstract

Caprine beta-mannosidosis is an autosomal recessive defect of glycoprotein catabolism with a deficiency of tissue and plasma beta-mannosidase activity and tissue accumulation of oligosaccharides within lysosomes. This rapidly fatal genetic disorder of Nubian goats is expressed at birth by a variety of clinical signs including deafness. Affected goats had folded pinnas, and the tympanic cavity was decreased due to multiple, polypoid projections of bone covered by middle ear mucosa which obstructed the view of the cochlear promontory. Numerous cells of the cochlear duct including mesothelial and epithelial cells of Reissner's membrane, mesothelial cells lining the scala tympani, cells of the stria vascularis, numerous supportive cells of the organ of Corti, cochlear hair cells, endothelial cells, perithelial cells, fibroblasts, macrophages, and neurons of the spiral ganglion contained numerous nonstaining intracytoplasmic vacuoles which resulted in distention of affected cells and caused thickening of involved structures. Ultrastructurally, the vacuoles were membrane-bound and consistent with lysosomes. Vacuolated cells were desquamated into the scala vestibuli and scala tympani. This is one of few reports describing light and el...Continue Reading

References

Jan 1, 1977·Archives of Neurology·J P KistlerR Dickersin
Jul 1, 1977·Medicine·J VidgoffN R Buist
Mar 22, 1986·The Veterinary Record·K KumarK L Lovell
Nov 6, 1986·The New England Journal of Medicine·D A WengerJ N Thompson
Jan 1, 1987·International Journal of Developmental Neuroscience : the Official Journal of the International Society for Developmental Neuroscience·K L Lovell, P J Boyer
Jan 1, 1985·Acta Neuropathologica·K L Lovell, M Z Jones
Jan 1, 1968·Archiv für klinische und experimentelle Ohren- Nasen- und Kehlkopfheilkunde·G Zechner, F Altmann
Nov 1, 1973·Acta paediatrica Scandinavica·S AutioT Louhimo
Jan 1, 1983·Acta Neuropathologica·K L Lovell, M Z Jones
Mar 1, 1983·Acta Oto-laryngologica·E Bichler, M Wieser
May 1, 1983·Journal of Neuropathology and Experimental Neurology·M Z JonesK L Lovell
Nov 1, 1981·Australian Veterinary Journal·P F HealyC A Sewell

❮ Previous
Next ❯

Citations

Jan 1, 1992·Biochimie·F PercheronB Ricard
Jan 1, 1992·Journal of Veterinary Diagnostic Investigation : Official Publication of the American Association of Veterinary Laboratory Diagnosticians, Inc·J A RenderJ F Hunter
Jan 1, 1990·Journal of Inherited Metabolic Disease·A CooperI B Sardharwalla

❮ Previous
Next ❯

Related Concepts

Trending Feeds

COVID-19

Coronaviruses encompass a large family of viruses that cause the common cold as well as more serious diseases, such as the ongoing outbreak of coronavirus disease 2019 (COVID-19; formally known as 2019-nCoV). Coronaviruses can spread from animals to humans; symptoms include fever, cough, shortness of breath, and breathing difficulties; in more severe cases, infection can lead to death. This feed covers recent research on COVID-19.

Blastomycosis

Blastomycosis fungal infections spread through inhaling Blastomyces dermatitidis spores. Discover the latest research on blastomycosis fungal infections here.

Nuclear Pore Complex in ALS/FTD

Alterations in nucleocytoplasmic transport, controlled by the nuclear pore complex, may be involved in the pathomechanism underlying multiple neurodegenerative diseases including Amyotrophic Lateral Sclerosis and Frontotemporal Dementia. Here is the latest research on the nuclear pore complex in ALS and FTD.

Applications of Molecular Barcoding

The concept of molecular barcoding is that each original DNA or RNA molecule is attached to a unique sequence barcode. Sequence reads having different barcodes represent different original molecules, while sequence reads having the same barcode are results of PCR duplication from one original molecule. Discover the latest research on molecular barcoding here.

Chronic Fatigue Syndrome

Chronic fatigue syndrome is a disease characterized by unexplained disabling fatigue; the pathology of which is incompletely understood. Discover the latest research on chronic fatigue syndrome here.

Evolution of Pluripotency

Pluripotency refers to the ability of a cell to develop into three primary germ cell layers of the embryo. This feed focuses on the mechanisms that underlie the evolution of pluripotency. Here is the latest research.

Position Effect Variegation

Position Effect Variagation occurs when a gene is inactivated due to its positioning near heterochromatic regions within a chromosome. Discover the latest research on Position Effect Variagation here.

STING Receptor Agonists

Stimulator of IFN genes (STING) are a group of transmembrane proteins that are involved in the induction of type I interferon that is important in the innate immune response. The stimulation of STING has been an active area of research in the treatment of cancer and infectious diseases. Here is the latest research on STING receptor agonists.

Microbicide

Microbicides are products that can be applied to vaginal or rectal mucosal surfaces with the goal of preventing, or at least significantly reducing, the transmission of sexually transmitted infections. Here is the latest research on microbicides.

Related Papers

Clinica Chimica Acta; International Journal of Clinical Chemistry
L BurdittB Winchester
Biochimica Et Biophysica Acta
J H LaBadie, N N Aronson
Clinica Chimica Acta; International Journal of Clinical Chemistry
M BernardF Percheron
© 2021 Meta ULC. All rights reserved