Axonal degeneration in paraplegin-deficient mice is associated with abnormal mitochondria and impairment of axonal transport

Ferreirinha, Fátima; Quattrini, Angelo; Pirozzi, Marinella; Valsecchi, Valentina; Dina, Giorgia; Broccoli, Vania; Auricchio, Alberto; Piemonte, Fiorella; Tozzi, Giulia; Gaeta, Laura; Casari, Giorgio; Ballabio, Andrea; Rugarli, Elena I.

doi:10.1172/jci20138

Cited by 128 publications

(88 citation statements)

References 47 publications

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“…SPG7 and SPG4 mice models develop axonal swellings filled with MBOs and cytoskeletal proteins (Tarrade et al, 2006). Injection of neurotracer into gastrocnemius muscle of SPG7 mice further demonstrated that retrograde AT is reduced in these mice (Ferreirinha et al, 2004). …”

Section: Hereditary Spastic Paraplegiasmentioning

confidence: 99%

See 1 more Smart Citation

Axonal Transport Defects in Neurodegenerative Diseases

Morfini¹,

Burns²,

Binder³

et al. 2009

J. Neurosci.

404

380

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Adult-onset neurodegenerative diseases (AONDs) comprise a heterogeneous group of neurological disorders characterized by a progressive, age-dependent decline in neuronal function and loss of selected neuronal populations. Alterations in synaptic function and axonal connectivity represent early and critical pathogenic events in AONDs, but molecular mechanisms underlying these defects remain elusive. The large size and complex subcellular architecture of neurons render them uniquely vulnerable to alterations in axonal transport (AT). Accordingly, deficits in AT have been documented in most AONDs, suggesting a common defect acquired through different pathogenic pathways. These observations suggest that many AONDs can be categorized as dysferopathies, diseases in which alterations in AT represent a critical component in pathogenesis. Topics here address various molecular mechanisms underlying alterations in AT in several AONDs. Illumination of such mechanisms provides a framework for the development of novel therapeutic strategies aimed to prevent axonal and synaptic dysfunction in several major AONDs.

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Section: Hereditary Spastic Paraplegiasmentioning

confidence: 99%

“…Mouse models of SPG7 and SPG4 provide in vivo evidence for AT impairment in HSPs. SPG7 is associated with mutations in paraplegin, a mitochondrial ATPase (Ferreirinha et al, 2004). SPG4 results from mutations in spastin, a microtubule severing protein (Baas et al, 2005).…”

Section: Hereditary Spastic Paraplegiasmentioning

confidence: 99%

Axonal Transport Defects in Neurodegenerative Diseases

Morfini¹,

Burns²,

Binder³

et al. 2009

J. Neurosci.

404

380

View full text Add to dashboard Cite

show abstract

“…The majority of their movements are microtubule-based, either anterograde via kinesins, toward the (+)-ends of the microtubules such as in axonal terminal, or retrograde via dynein, toward the (−)-ends in the cell bodies (Chang et al, 2006; Hollenbeck and Saxton, 2005; Overly et al, 1996). It is not surprising, therefore, that the dynamic nature of mitochondria is significant for neurons to maintain neuronal function and plasticity, and the abnormalities in mitochondrial distribution have been found in many neurodegenerative disease models such as Charcot-Marie-Tooth (Baloh et al, 2007), Amyotrophic Lateral Sclerosis (De Vos et al, 2007), Alzheimer's (Pigino et al, 2003; Rui et al, 2006; Thies and Mandelkow, 2007), Huntington's (Trushina et al, 2004), and Hereditary Spastic Paraplegia (Ferreirinha et al, 2004; McDermott et al, 2003). To understand the means by which mitochondrial distribution is achieved and the dynamic regulation of that distribution, static imagery is insufficient.…”

Section: Introductionmentioning

confidence: 99%

Chapter 18 Imaging Axonal Transport of Mitochondria

Wang

Schwarz

2009

Methods in Enzymology

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Neuronal mitochondria need to be transported and distributed in axons and dendrites in order to ensure an adequate energy supply and provide sufficient Ca 2+ buffering in each portion of these highly extended cells. Errors in mitochondrial transport are implicated in neurodegenerative diseases. Here we present useful tools to analyze axonal transport of mitochondria both in vitro in cultured rat neurons and in vivo in Drosophila larval neurons. These methods enable investigators to take advantage of both systems to study the properties of mitochondrial motility under normal or pathological conditions.

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“…61 A defective respiration seems to be particularly deleterious to long axons, possibly by affecting the energy demanding anterograde and retrograde axonal transport. 63 …”

Section: Disorders Involving Other Metalloproteasesmentioning

confidence: 99%

The genetic and molecular bases of monogenic disorders affecting proteolytic systems

Richard¹

2005

Journal of Medical Genetics

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Axonal degeneration in paraplegin-deficient mice is associated with abnormal mitochondria and impairment of axonal transport

Cited by 128 publications

References 47 publications

Axonal Transport Defects in Neurodegenerative Diseases

Axonal Transport Defects in Neurodegenerative Diseases

Chapter 18 Imaging Axonal Transport of Mitochondria

The genetic and molecular bases of monogenic disorders affecting proteolytic systems

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