Mice expressing the alpha(1B)-adrenergic receptor induces a synucleinopathy with excessive tyrosine nitration but decreased phosphorylation

J Neurochem. 2002 Nov;83(3):623-34. doi: 10.1046/j.1471-4159.2002.01170.x.

Abstract

We had previously reported that systemic overexpression of the alpha(1B)-adrenergic receptor (AR) in a transgenic mouse induced a neurodegenerative disease that resembled the parkinsonian-like syndrome called multiple system atrophy (MSA). We now report that our mouse model has cytoplasmic inclusion bodies that colocalize with oligodendrocytes and neurons, are positive for alpha-synuclein and ubiquitin, and therefore may be classified as a synucleinopathy. Alpha-synuclein monomers as well as multimers were present in brain extracts from both normal and transgenic mice. However, similar to human MSA and other synucleinopathies, transgenic mice showed an increase in abnormal aggregated forms of alpha-synuclein, which also increased its nitrated content with age. However, the same extracts displayed decreased phosphorylation of alpha-synuclein. Other traits particular to MSA such as Purkinje cell loss in the cerebellum and degeneration of the intermediolateral cell columns of the spinal cord also exist in our mouse model but differences still exist between them. Interestingly, long-term therapy with the alpha(1)-AR antagonist, terazosin, resulted in protection against the symptomatic as well as the neurodegeneration and alpha-synuclein inclusion body formation, suggesting that signaling of the alpha(1B)-AR is the cause of the pathology. We conclude that overexpression of the alpha(1B)-AR can cause a synucleinopathy similar to other parkinsonian syndromes.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Adrenergic alpha-Antagonists / pharmacology
  • Animals
  • Body Weight
  • Brain / pathology
  • Cerebellum / pathology
  • Disease Models, Animal
  • Female
  • Inclusion Bodies / drug effects
  • Inclusion Bodies / metabolism
  • Inclusion Bodies / pathology
  • Macromolecular Substances
  • Male
  • Mice
  • Mice, Transgenic
  • Motor Activity / genetics
  • Multiple System Atrophy / drug therapy
  • Multiple System Atrophy / genetics
  • Multiple System Atrophy / metabolism
  • Multiple System Atrophy / pathology
  • Nerve Tissue Proteins / biosynthesis*
  • Neurodegenerative Diseases / drug therapy
  • Neurodegenerative Diseases / genetics
  • Neurodegenerative Diseases / metabolism*
  • Neurodegenerative Diseases / pathology
  • Neurons / drug effects
  • Neurons / pathology
  • Nitrates / metabolism*
  • Oligodendroglia / pathology
  • Phosphorylation
  • Prazosin / analogs & derivatives*
  • Prazosin / pharmacology
  • Receptors, Adrenergic, alpha-1 / biosynthesis*
  • Receptors, Adrenergic, alpha-1 / genetics
  • Spinal Cord / pathology
  • Survival Rate
  • Synucleins
  • Tyrosine / metabolism*
  • Ubiquitin / biosynthesis
  • alpha-Synuclein

Substances

  • ADRA1B protein, human
  • Adra1b protein, mouse
  • Adrenergic alpha-Antagonists
  • Macromolecular Substances
  • Nerve Tissue Proteins
  • Nitrates
  • Receptors, Adrenergic, alpha-1
  • Snca protein, mouse
  • Synucleins
  • Ubiquitin
  • alpha-Synuclein
  • Tyrosine
  • Terazosin
  • Prazosin