Alexander’s disease in a bernese mountain dog

Weissenböck, Herbert; Obermaier, Gabriele; Dahme, Erwin

doi:10.1007/s004010050414

Cited by 19 publications

(19 citation statements)

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“…Such perivascular lymphocytic infiltration has also been reported in human AD [15], although there have been no information about the change in previous canine AD-like cases [1,2,7,12,18]. The evidence of blood-brain-barrier (BBB) disruption has been confirmed by magnetic resonance imaging examinations using contrast-enhancement in human AD patients [9].…”

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confidence: 85%

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Fibrinoid Leukodystrophy (Alexander's Disease-Like Disorder) in a Young Adult French Bulldog

Ito

Uchida

Nakamura

et al. 2010

The Journal of Veterinary Medical Science

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ABSTRACT. The paper describes clinical and pathological features of Alexander's disease (AD)-like disorder in a 1 year and 8 months old French bulldog. Clinically, the dog exhibited megaesophagus, emaciation and weakness without any specific neurological symptoms. The dog died of aspiration pneumonia. On the gross observation of formalin-fixed brain, discolored foci were observed in the white matter of the cerebellum and brain stem. Histologically, numerous Rothenthal fibers and hypertrophic astrocytes were distributed especially in the perivascular, subependymal and subpial area of both the cerebrum and cerebellum. The Rosenthal fibers were intensely immunopositive for GFAP and ubiquitin. Demyelination of the white matter was occasionally found in the brain stem. The present case is likely to be categorized in the adult form of AD, though previous AD-like cases in dogs were in the juvenile form.

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confidence: 85%

“…All of the dog cases [1,2,7,12,18] developed the AD-like disorders by 6-month-old, thus they were considered to resemble the juvenile form of AD (Table 1). The present paper describes a caine case of adult-onset AD.…”

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confidence: 99%

Fibrinoid Leukodystrophy (Alexander's Disease-Like Disorder) in a Young Adult French Bulldog

Ito

Uchida

Nakamura

et al. 2010

The Journal of Veterinary Medical Science

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“…The Rosenthal fibres characteristic of Alexander disease constitute abnormal inclusions containing Bc (some of which is ubiquitinated) [174], Hsp27 and GAFP [175,176]. The involvement of Hsp27 and Bc in the formation of Rosenthal fibres within astrocytes is believed to be a response to an as-yet unknown stress caused by the disease [77].…”

Section: The Role Of Shsps In Neurodegenerative Diseasementioning

confidence: 99%

Small heat-shock proteins: important players in regulating cellular proteostasis

Treweek

Meehan

Ecroyd

et al. 2014

Cell. Mol. Life Sci.

180

177

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Small heat-shock proteins (sHsps) are a diverse family of intra-cellular molecular chaperone proteins that play a critical role in mitigating and preventing protein aggregation under stress conditions such as elevated temperature, oxidation and infection. In doing so, they assist in the maintenance of protein homeostasis (proteostasis) thereby avoiding the deleterious effects that result from loss of protein function and/or protein aggregation. The chaperone properties of sHsps are therefore employed extensively in many tissues to prevent the development of diseases associated with protein aggregation. Significant progress has been made of late in understanding the structure and chaperone mechanism of sHsps. In this review, we discuss some of these advances, with a focus on mammalian sHsp hetero-oligomerisation, the mechanism by which sHsps act as molecular chaperones to prevent both amorphous and fibrillar protein aggregation, and the role of posttranslational modifications in sHsp chaperone function, particularly in the context of disease. 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 35 36 37 38 39 40 41 42 43 44 45 46 47 48 49 50 51 52 53 54 55 56 57 58 59 60 61 62 63 64 65 2 Abstract (word count = 159)Small heat-shock proteins (sHsps) are a diverse family of intracellular molecular chaperone proteins that play a critical role in preventing protein unfolding, misfolding and aggregation, particularly under stress conditions such as elevated temperature, oxidation and infection. In doing so, they assist in the maintenance of protein homeostasis (proteostasis) and thereby avoid the deleterious effects that result from loss of protein function and/or protein aggregation. The chaperone properties of sHsps are therefore employed extensively in many tissues to prevent the development of diseases associated with protein aggregation. There has been much research into the structure and mechanism of chaperone action of sHsps over approximately the past 30 years, and significant progress has been made of late, however, there are still many unanswered questions relating to these aspects of sHsps. In this review, we outline some of the recent advances in understanding the structure and function of mammalian sHsps, particularly in the context of their many and varied roles in disease.

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“…3,4,6 In animals, RFs have very rarely been observed, being only reported in cases clinically and morphologically identical to AD in humans. They have been described in 5 dogs of different breeds (2 Labrador retrievers, 1 Scottish terrier, 1 Miniature poodle, 1 Bernese mountain dog) 1,5,7,8,10 and in 1 sheep. 2 As has been suggested by other authors, 9 if we take into account the age at onset and the type and topography of lesions in the central nervous system, all canine cases, including this dog, could be considered juvenile forms of AD, whereas the adult sheep that showed RFs and limited myelin changes could be viewed as an adult-onset form of AD.…”

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Rosenthal Fiber Encephalopathy in a Dog Resembling Alexander Disease in Humans

Alemañ¹,

Marcaccini²,

Espino³

et al. 2006

Vet Pathol

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Abstract. A young male Bernese mountain dog presented with neurologic abnormalities consisting of nonambulatory tetraparesis, generalized tremors, and depressed mental status. At necropsy only a mild enlargement of the lateral ventricles was seen. The histologic examination revealed the presence of eosinophilic deposits consistent with Rosenthal fibers (RFs) throughout the white matter of the central nervous system. There was also a marked proliferation of abnormally large astrocytes and limited myelin changes. RFs were most prominent in perivascular, subpial, and subependymal areas, where they were perpendicularly located, producing a pallisaded arrangement. Immunohistochemically, RFs were strongly positive for glial fibrillary acidic protein (GFAP), and when they were examined ultrastructurally they appeared as electron-dense amorphous masses located within the processes of astrocytes, most particularly in the perivascular feet. The histologic and immunohistochemical findings of this canine case were consistent with the published neuropathologic descriptions of Alexander disease in humans and in a few dogs, a rare condition that in humans has been shown to be caused by dominant mutations in the GFAP gene.Key words: Alexander disease; Bernese mountain dog; encephalopathy; GFAP; leukodystrophy; Rosenthal fibers; transmission electron microscopy.A 4-month-old male Bernese mountain dog presented to the Hospital Veterinario Rof Codina with a 3-week history of progressive tetraparesis and tremors, particularly of the hind limbs. The dog had been treated with prednisone (Dacortin, Merck Farma y Química, S. A., Barcelona, Spain) at 0.5 mg/kg per os twice a day for 3 days, but it did not respond to the therapy and the condition worsened rapidly. On admission, the neurologic examination revealed nonambulatory tetraparesis, generalized tremors and depressed mental status. Postural reactions were difficult to evaluate because of generalized weakness, but they were considered to be abnormally slow. Spinal cord reflexes were decreased in all 4 limbs, but cranial nerve function and muscle tone were preserved. The laboratory data revealed normal urine and blood values. A multifocal involvement of the central nervous system was suspected, and, therefore, sampling of cerebrospinal fluid (CSF) was performed by cerebellomedullary cisternal puncture. CSF analysis showed 5 cells/ml (reference range, ,8 cells/ml), total protein concentration of 21 mg/dl (reference range, ,25 mg/dl), and absence of neutralizing antibodies against canine distemper virus. Because of the deteriorated neurologic status, and at the request of the owner, the dog was euthanatized, and a complete necropsy was performed.Gross lesions were found only in the brain, with a moderate enlargement of the lateral ventricles. The brain and the cranial portion of the cervical spinal cord were fixed by immersion in 10% buffered formalin, and then representative coronal slices were embedded in paraffin according to standard laboratory procedures.Paraffin sections (4-5 mm...

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Alexander’s disease in a bernese mountain dog

Cited by 19 publications

References 19 publications

Fibrinoid Leukodystrophy (Alexander's Disease-Like Disorder) in a Young Adult French Bulldog

Fibrinoid Leukodystrophy (Alexander's Disease-Like Disorder) in a Young Adult French Bulldog

Small heat-shock proteins: important players in regulating cellular proteostasis

Rosenthal Fiber Encephalopathy in a Dog Resembling Alexander Disease in Humans

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