Amyotrophic Lateral Sclerosis:Mammalian Cell Models,Copper-Zinc Superoxide Dismutaseand Biological Characteristics

"Amyotrophic Lateral Sclerosis (ALS) is the most severe and common adult onset disorder that affects motor neurons in the spinal cord, brainstem and cortex, resulting in progressive weakness and death from respiratory failure within two to five years of symptoms onset(...)

Haematophagic behavior in laboratory of Lutzomyia cruzi (Mangabeira) (Diptera: Psychodidae) in relation to three mammalian blood sources in Manaus, Brazil

The sand fly Lutzomyia cruzi is considered as one of vectors of visceral leishmaniasis in Brazil. This work examined optimum feeding age, feeding time, host preference, fecundity rates, and female blood meal volume taken by single females from a closed colony of L. cruzi. Mean feeding time was longer on hamsters, 6.6 minutes, than on humans, 5.7 minutes. 49.1% of the 48h-old flies fed on humans and 43.3% of 72h-old flies fed on hamsters. Of a total of 120 females, 61% fed on humans and 25% fed on hamsters. Total fecundity was significantly higher in females fed on hamster than on human or opossum. Laboratory-reared L. cruzi females fed earlier, more promptly, and preferably on humans than on hamsters when offered these blood-meal sources simultaneously. The blood-meal volume is higher in females fed on hamsters than other hosts (human and opossum).

The role of the mammalian DNA end-processing enzyme polynucleotide kinase 3'-phosphatase in spinocerebellar ataxia Type 3 pathogenesis

DNA strand-breaks (SBs) with non-ligatable ends are generated by ionizing radiation, oxidative stress, various chemotherapeutic agents, and also as base excision repair (BER) intermediates. Several neurological diseases have already been identified as being due to a deficiency in DNA end-processing activities. Two common dirty ends, 3'-P and 5'-OH, are processed by mammalian polynucleotide kinase 3'-phosphatase (PNKP), a bifunctional enzyme with 3'-phosphatase and 5'-kinase activities. We have made the unexpected observation that PNKP stably associates with Ataxin-3 (ATXN3), a polyglutamine repeat-containing protein mutated in spinocerebellar ataxia type 3 (SCA3), also known as Machado-Joseph Disease (MJD). This disease is one of the most common dominantly inherited ataxias worldwide; the defect in SCA3 is due to CAG repeat expansion (from the normal 14-41 to 55-82 repeats) in the ATXN3 coding region. However, how the expanded form gains its toxic function is still not clearly understood. Here we report that purified wild-type (WT) ATXN3 stimulates, and by contrast the mutant form specifically inhibits, PNKP's 3' phosphatase activity in vitro. ATXN3-deficient cells also show decreased PNKP activity. Furthermore, transgenic mice conditionally expressing the pathological form of human ATXN3 also showed decreased 3'-phosphatase activity of PNKP, mostly in the deep cerebellar nuclei, one of the most affected regions in MJD patients' brain. Finally, long amplicon quantitative PCR analysis of human MJD patients' brain samples showed a significant accumulation of DNA strand breaks. Our results thus indicate that the accumulation of DNA strand breaks due to functional deficiency of PNKP is etiologically linked to the pathogenesis of SCA3/MJD.

Protoptychus hatcheri Scott, 1895 : the mammalian faunas of the Washakie Formation, Eocene age, of southern Wyoming. William D. Turnbull --.

n.s. no.21(1991)

The mammalian faunas of the Washakie Formation, Eocene age, of southern Wyoming. Steven M. McCarroll, John J. Flynn, William D. Turnbull --.

n.s. no.33(1996)

The mammalian fauna of Madura Cave, Western Australia. Macropodidae: Potoroinae / Ernest L. Lundelius, Jr. -- William D. Turnbull --.

n.s. no.14(1984)

The mammalian fauna of Madura Cave, Western Australia, Ernest L. Lundelius, Jr. -- William D. Turnbull --.

n.s. no.6(1981)

Mammalian diversity on Mount Isarog : a threatened center of endemism on southern Luzon Island, Philippines / Lawrence R. Heaney ... [et al.].

n.s. no.95(1999)

The mammalian fauna of Madura Cave, Western Australia : Ernest L. Lundelius, Jr. -- and William D. Turnbull --.

v.31:no.1(1973)

The mammalian fauna of Madura Cave, Western Australia. Ernest L. Lundelius, Jr. -- William D. Turnbull --.

n.s. no.17(1989)

A synopsis of the mammalian fauna of the Philippine Islands / Lawrence R. Heaney ... [et al.].

n.s. no.88(1998)

The mammalian fauna of Madura Cave, Western Australia : Ernest L. Lundelius, Jr. -- and William D. Turnbull --.

v.38(1978)