Spinal Muscular Atrophy & Neuron Regeneration

Below are the top 20 results from the following PubMed search, sorted by “Best Match,” limited to the past 10 years: “neural regeneration” OR “neuron regeneration” OR “nerve regeneration”[Title/Abstract:~0]

(Last updated March 2023)

Characteristics and advantages of adeno-associated virus vector-mediated gene therapy for neurodegenerative diseases.

Qu Y, Liu Y, Noor AF, Tran J, Li R.

Neural Regen Res. 2019 Jun;14(6):931-938. doi: 10.4103/1673-5374.250570.

PMID: 30761996 Free PMC article. Review.

 

 

RNA transport and localized protein synthesis in neurological disorders and neural repair.

Wang W, van Niekerk E, Willis DE, Twiss JL.

Dev Neurobiol. 2007 Aug;67(9):1166-82. doi: 10.1002/dneu.20511.

PMID: 17514714 Review.

Neural cells are able to finely tune gene expression through post-transcriptional mechanisms. …Activity of the translational machinery is also regulated in distal neural cell processes. Clinically, disruption of mRNA localization and/or localized mRNA translation …

 

 

Nerve sprouting capacity in a pharmacologically induced mouse model of spinal muscular atrophy.

Rimer M, Seaberg BL, Yen PF, Lam S, Hastings RL, Lee YI, Thompson WJ, Feng Z, Metzger F, Paushkin S, Ko CP.

Sci Rep. 2019 May 24;9(1):7799. doi: 10.1038/s41598-019-44222-2.

PMID: 31127156 Free PMC article.

Spinal muscular atrophy (SMA) is caused by loss-of-function mutations in the survival of motoneuron gene 1 (SMN1). …At suboptimal doses, splicing modifiers, such as SMN-C1, have served to generate mice that model milder SMA, referred to as pharmacological S …

 

 

Gene therapy for peripheral nervous system diseases.

Federici T, Boulis N.

Curr Gene Ther. 2007 Aug;7(4):239-48. doi: 10.2174/156652307781369083.

PMID: 17969557 Review.

Currently, the treatment of peripheral neuropathies is more focused on managing pain rather than providing permissive conditions for regeneration. Despite advances in microsurgical techniques, including nerve grafting and reanastomosis, axonal regeneration af …

 

 

The clinical spectrum of SMA-PME and in vitro normalization of its cellular ceramide profile.

Lee MM, McDowell GSV, De Vivo DC, Friedman D, Berkovic SF, Spanou M, Dinopoulos A, Grand K, Sanchez-Lara PA, Allen-Sharpley M, Warman-Chardon J, Solyom A, Levade T, Schuchman EH, Bennett SAL, Dyment DA, Pearson TS.

Ann Clin Transl Neurol. 2022 Dec;9(12):1941-1952. doi: 10.1002/acn3.51687. Epub 2022 Nov 3.

PMID: 36325744 Free PMC article. Review.

OBJECTIVE: The objectives of this study were to define the clinical and biochemical spectrum of spinal muscular atrophy with progressive myoclonic epilepsy (SMA-PME) and to determine if aberrant cellular ceramide accumulation could be normalized by enzyme rep …

 

 

Prospective use of skin-derived precursors in neural regeneration.

Lu XC, Tao Y, Li LX.

Chin Med J (Engl). 2012 Dec;125(24):4488-96.

PMID: 23253725 Review.

The search terms used were “skin-derived precursors”, “stem cells”, and “neural diseases”. STUDY SELECTION: Articles were included in the review if they were relevant to SKPs as stem cells, as well as their applications in neural regenerative medicine, such as in th …

 

 

Defects in neuromuscular junction remodelling in the Smn(2B/-) mouse model of spinal muscular atrophy.

Murray LM, Beauvais A, Bhanot K, Kothary R.

Neurobiol Dis. 2013 Jan;49:57-67. doi: 10.1016/j.nbd.2012.08.019. Epub 2012 Aug 30.

PMID: 22960106

Spinal muscular atrophy (SMA) is a devastating childhood motor neuron disease caused by mutations and deletions within the survival motor neuron 1 (SMN1) gene. …We show that whilst NMJs are capable of remodelling during pathogenesis, there is …

 

 

Biodegradable chitin conduit tubulation combined with bone marrow mesenchymal stem cell transplantation for treatment of spinal cord injury by reducing glial scar and cavity formation.

Xue F, Wu EJ, Zhang PX, Li-Ya A, Kou YH, Yin XF, Han N.

Neural Regen Res. 2015 Jan;10(1):104-11. doi: 10.4103/1673-5374.150715.

PMID: 25788929 Free PMC article.

We examined the restorative effect of modified biodegradable chitin conduits in combination with bone marrow mesenchymal stem cell transplantation after right spinal cord hemisection injury. Immunohistochemical staining revealed that biological conduit sleeve bridging redu …

 

 

Peripheral nerve involvement in Werdnig-Hoffmann disease.

Chien YY, Nonaka I.

Brain Dev. 1989;11(4):221-9. doi: 10.1016/s0387-7604(89)80040-3.

PMID: 2774090

The number of large myelinated axons was markedly decreased in almost all the intramuscular nerve bundles included in 32 muscle biopsies from patients with Werdnig-Hoffmann disease compared to that in normals. …The earlier damage to the peripheral nerves probably resulte …

 

 

Activity-driven synaptic and axonal degeneration in canine motor neuron disease.

Carrasco DI, Rich MM, Wang Q, Cope TC, Pinter MJ.

J Neurophysiol. 2004 Aug;92(2):1175-81. doi: 10.1152/jn.00157.2004. Epub 2004 Mar 17.

PMID: 15028742 Free article.

The role of neuronal activity in the pathogenesis of neurodegenerative disease is largely unknown. In this study, we examined the effects of increasing motor neuron activity on the pathogenesis of a canine version of inherited motor neuron disease (hereditary canine …

 

 

A comparison of fibre size, fibre type constitution and spatial fibre type distribution in normal human muscle and in muscle from cases of spinal muscular atrophy and from other neuromuscular disorders.

Johnson MA, Sideri G, Weightman D, Appleton D.

J Neurol Sci. 1973 Dec;20(4):345-61. doi: 10.1016/0022-510x(73)90169-x.

PMID: 4272515 No abstract available.

 

 

Carnitine and acyltransferase in experimental neurogenic atrophies: changes with treatment.

Bresolin N, Freddo L, Tegazzin V, Bet L, Armani M, Angelini C.

J Neurol. 1984;231(4):170-5. doi: 10.1007/BF00313933.

PMID: 6512569

Carnitine level and carnitine palmityl transferase (CPT) activity were investigated in muscles of patients with infantile and juvenile spinal muscular atrophy and polyneuropathies. A significant decrease of both carnitine and CPT was found in the infantile …

 

 

Widespread electromyographic abnormalities in patients with monomelic amyotrophy: a detailed EMG study.

Khandelwal D, Bhatia M, Singh S, Shukla G, Goyal V, Srivastava T, Behari M.

Electromyogr Clin Neurophysiol. 2005 Sep-Oct;45(6):363-7.

PMID: 16315974

OBJECTIVE: To evaluate subclinical electromyographic changes in unaffected muscles of the patients with monomelic amyotrophy (MMA). PATIENTS AND METHODS: 35 patients of MMA with single limb atrophy (30 patients with upper limb involvement and 5 patients with lower limb inv …

 

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