Kenneth N Maclean
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Explore the profile of Kenneth N Maclean including associated specialties, affiliations and a list of published articles.
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61
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1223
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Recent Articles
1.
Maclean K, Jiang H, Neill P, Chanin R, Hurt K, Orlicky D, et al.
FASEB J
. 2024 Jul;
38(13):e23795.
PMID: 38984928
Cystathionine beta-synthase-deficient homocystinuria (HCU) is a life-threatening disorder of sulfur metabolism. HCU can be treated by using betaine to lower tissue and plasma levels of homocysteine (Hcy). Here, we show...
2.
Swanson M, Jiang H, Busquet N, Carlsen J, Brindley C, Benke T, et al.
J Inherit Metab Dis
. 2024 Jun;
47(5):971-990.
PMID: 38840294
Nonketotic hyperglycinemia due to deficient glycine cleavage enzyme activity causes a severe neonatal epileptic encephalopathy. Current therapies based on mitigating glycine excess have only limited impact. An animal model with...
3.
Swanson M, Jiang H, Busquet N, Carlsen J, Brindley C, Benke T, et al.
bioRxiv
. 2024 Apr;
PMID: 38586005
Take Home Message: A mouse model of nonketotic hyperglycinemia is described that shows postnatal abnormalities in glycine levels, neural tube defects, body weight, electroencephalographic recordings, and in activity in young...
4.
Yousof T, Bouchard C, Alb M, Lynn E, Lhotak S, Jiang H, et al.
J Biol Chem
. 2024 Jan;
300(2):105655.
PMID: 38237682
Endoplasmic reticulum stress is associated with insulin resistance and the development of nonalcoholic fatty liver disease. Deficiency of the endoplasmic reticulum stress response T-cell death-associated gene 51 (TDAG51) (TDAG51) in...
5.
Prutton K, Marentette J, Maclean K, Roede J
Free Radic Biol Med
. 2023 Jan;
196:11-21.
PMID: 36638900
Cellular redox state directs differentiation of induced pluripotent stem cells (iPSC) by energy metabolism control and ROS generation. As oxidative stress and mitochondrial dysfunction have been extensively reported in Down...
6.
Prutton K, Marentette J, Leifheit B, Esquer H, LaBarbera D, Anderson C, et al.
Free Radic Biol Med
. 2022 May;
186:32-42.
PMID: 35537597
The ubiquity of cognitive deficits and early onset Alzheimer's disease in Down syndrome (DS) has focused much DS iPSC-based research on neuron degeneration and regeneration. Despite reports of elevated oxidative...
7.
Anderson C, Marentette J, Prutton K, Rauniyar A, Reisz J, DAlessandro A, et al.
Free Radic Biol Med
. 2021 Jun;
172:201-212.
PMID: 34129926
Down syndrome (DS) is the most common genetic cause of intellectual disability. Mechanistically, oxidative stress and mitochondrial dysfunction are reported to be etiological factors for many of the DS-related comorbidities...
8.
Gupta S, Wang L, Slifker M, Cai K, Maclean K, Wasek B, et al.
FASEB J
. 2021 May;
35(6):e21629.
PMID: 33949005
Cystathionine beta-synthase (CBS) is a key enzyme of the trans-sulfuration pathway that converts homocysteine to cystathionine. Loss of CBS activity due to mutation results in CBS deficiency, an inborn error...
9.
Bok R, Guerra D, Lorca R, Wennersten S, Harris P, Rauniyar A, et al.
Redox Biol
. 2021 Jan;
40:101827.
PMID: 33485059
During pregnancy, estrogen (E) stimulates uterine artery blood flow (UBF) by enhancing nitric oxide (NO)-dependent vasodilation. Cystathionine γ-lyase (CSE) promotes vascular NO signaling by producing hydrogen sulfide (HS) and by...
10.
Maclean K, Jiang H, Phinney W, Mclagan B, Roede J, Stabler S
Mol Genet Metab
. 2021 Jan;
132(2):128-138.
PMID: 33483253
Cystathionine beta-synthase deficient homocystinuria (HCU) is a life-threatening disorder of sulfur metabolism. Our knowledge of the metabolic changes induced in HCU are based almost exclusively on data derived from plasma....