Adrian G Todd
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Explore the profile of Adrian G Todd including associated specialties, affiliations and a list of published articles.
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19
Citations
241
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Recent Articles
1.
Fuller D, Rana S, Thakre P, Benevides E, Pope M, Todd A, et al.
bioRxiv
. 2025 Jan;
PMID: 39763722
Absence of functional acid-α-glucosidase (GAA) leads to early-onset Pompe disease with cardiorespiratory and neuromuscular failure. A novel Pompe rat model ( ) was used to test the hypothesis that neonatal...
2.
Chittoor-Vinod V, Bazick H, Todd A, Falk D, Morelli K, Burgess R, et al.
ACS Chem Neurosci
. 2019 Apr;
10(6):2890-2902.
PMID: 31017387
Hereditary demyelinating neuropathies linked to peripheral myelin protein 22 (PMP22) involve the disruption of normal protein trafficking and are therefore relevant targets for chaperone therapy. Using a small molecule HSP90...
3.
Falk D, Galatas T, Todd A, Soto E, Harris A, Notterpek L
Muscle Nerve
. 2017 Oct;
57(4):664-671.
PMID: 29023846
Introduction: Patients with hereditary peripheral neuropathies exhibit characteristic deformities of the hands and feet and have difficulty ambulating. To examine to what extent neuropathic animals recapitulate these deficits, we studied...
4.
Baligand C, Todd A, Lee-McMullen B, Vohra R, Byrne B, Falk D, et al.
Mol Ther Methods Clin Dev
. 2017 Oct;
7:42-49.
PMID: 29018835
The development of therapeutic clinical trials for glycogen storage disorders, including Pompe disease, has called for non-invasive and objective biomarkers. Glycogen accumulation can be measured in vivo with C MRS....
5.
Doerfler P, Todd A, Clement N, Falk D, Nayak S, Herzog R, et al.
Hum Gene Ther
. 2015 Nov;
27(1):43-59.
PMID: 26603344
Pompe disease is a progressive neuromuscular disorder caused by lysosomal accumulation of glycogen from a deficiency in acid alpha-glucosidase (GAA). Replacement of the missing enzyme is available by repeated protein...
6.
Todd A, McElroy J, Grange R, Fuller D, Walter G, Byrne B, et al.
Ann Neurol
. 2015 May;
78(2):222-34.
PMID: 25925726
Objective: We have recently reported on the pathology of the neuromuscular junction (NMJ) in Pompe disease, reflecting disruption of neuronal and muscle homeostasis as a result of glycogen accumulation. The...
7.
Nicks J, Lee S, Harris A, Falk D, Todd A, Arredondo K, et al.
Neurobiol Dis
. 2014 Jul;
70:224-36.
PMID: 25014022
Charcot--Marie-Tooth disease type 1A (CMT1A) is a hereditary peripheral neuropathy characterized by progressive demyelination and distal muscle weakness. Abnormal expression of peripheral myelin protein 22 (PMP22) has been linked to...
8.
Custer S, Todd A, Singh N, Androphy E
Hum Mol Genet
. 2013 Jun;
22(20):4043-52.
PMID: 23727837
Spinal muscular atrophy (SMA) is a devastating neuromuscular disorder that stems from low levels of survival of motor neuron (SMN) protein. The processes that cause motor neurons and muscle cells...
9.
Todd A, Lin H, Ebert A, Liu Y, Androphy E
Hum Mol Genet
. 2012 Nov;
22(4):729-36.
PMID: 23175440
Our fundamental understanding of how several thousand diverse RNAs are recognized in the soma, sorted, packaged, transported and localized within the cell is fragmentary. The COPa and COPb proteins of...
10.
Morse R, Todd A, Young P
Methods Mol Biol
. 2012 Mar;
867:349-62.
PMID: 22454072
Many genetic mutations result in the disruption of (alternative) splicing. Prime examples are the SMN1 and SMN2 genes: a silent mutation in SMN2 leads to the skipping of the constitutive...