Numerous human diseases are caused by protein folding defects where the protein may become more susceptible to degradation or aggregation. Aberrant protein folding can affect the kinetic stability of the proteins even if these proteins appear to be soluble in vivo.
Scientific News
FARA funds research progress
In this section, you will find the most recent FA research publications, many of which are funded by FARA, as well as information on upcoming conferences and symposiums. You can search for articles by date using the archive box in the right hand column. To locate FARA Funded or Supported Research, click the hyperlink in the right hand column. You may also search for specific content using key words or phrases in the search button at the top right of your screen. Please be sure to visit other key research sections of our website for information on FARA's Grant Program and the Treatment Pipeline.
Probing the Kinetic Stabilities of Friedreich's Ataxia Clinical Variants Using a Solid Phase GroEL Chaperonin Capture Platform
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Comparing speech characteristics in spinocerebellar ataxias type 3 and type 6 with Friedreich ataxia
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Patterns of dysarthria in spinocerebellar ataxias (SCAs) and their discriminative features still remain elusive. Here we aimed to compare dysarthria profiles of patients with (SCA3 and SCA6 vs. Friedreich ataxia (FRDA), focussing on three particularly vulnerable speech parameters (speaking rate, prosodic modulation, and intelligibility) in ataxic dysarthria as well as on a specific oral non-speech variable of ataxic impairment, i.e., the irregularity of oral motor diadochokinesis (DDK).
Read More: Comparing speech characteristics in spinocerebellar ataxias type 3 and type 6 with Friedreich ataxia
Nasality in Friedreich ataxia
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Perceptual speech research in Friedreich ataxia (FRDA) has identified altered nasality as a key component of the dysarthria profile, however the incidence and severity of abnormal nasality remains unknown. Utilizing objective and perceptual methods, data on the relationship between resonance, disease duration, severity, age of onset and genetic profiles were collated.
Read More: Nasality in Friedreich ataxia
Overlapping Binding Sites of the Frataxin Homologue Assembly Factor
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In mitochondria FeS clusters, prosthetic groups critical for the activity of many proteins, are first assembled on Isu, a 14-kDa scaffold protein, and then transferred to recipient apoproteins. The assembly process involves interaction of Isu with both Nfs1, the cysteine desulfurase serving as a sulfur donor, and the yeast frataxin homolog (Yfh1) serving as a regulator of desulfurase activity and/or iron donor.
A longitudinal VBM study monitoring treatment with erythropoietin in patients with Friedreich ataxia
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BACKGROUND:
Recombinant human erythropoietin (rhuEPO) has received considerable attention because of its neuroprotective properties. It has recently been reported that rhuEPO increases frataxin levels in combination with clinical improvement in rhuEPO treated patients with Friedreich ataxia (FRDA).
Read More: A longitudinal VBM study monitoring treatment with erythropoietin in patients with Friedreich ataxia
- Iron-sulfur cluster biogenesis in mammalian cells: New insights into the molecular mechanisms of cluster delivery
- Understanding the frustration arising from the competition between function, misfolding, and aggregation in a globular protein
- Cellular, Molecular and Functional Characterisation of YAC Transgenic Mouse Models of Friedreich Ataxia
- Overlapping Binding Sites of the Frataxin Homologue Assembly Factor and the Heat Shock Protein 70 Transfer Factor on the Isu Iron-sulfur Cluster Scaffold Protein
- Frataxin activates Fe-S cluster biosynthesis by facilitating sulfur transfer chemistry
