Anil B Mukherjee
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Explore the profile of Anil B Mukherjee including associated specialties, affiliations and a list of published articles.
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46
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1149
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Recent Articles
1.
Bagh M, Appu A, Sadhukhan T, Mondal A, Plavelil N, Raghavankutty M, et al.
J Biol Chem
. 2024 Jan;
300(2):105641.
PMID: 38211816
The ceroid lipofuscinosis neuronal 1 (CLN1) disease, formerly called infantile neuronal ceroid lipofuscinosis, is a fatal hereditary neurodegenerative lysosomal storage disorder. This disease is caused by loss-of-function mutations in the...
2.
Mondal A, Appu A, Sadhukhan T, Bagh M, Previde R, Sadhukhan S, et al.
J Inherit Metab Dis
. 2022 Feb;
45(3):635-656.
PMID: 35150145
Inactivating mutations in the PPT1 gene encoding palmitoyl-protein thioesterase-1 (PPT1) underlie the CLN1 disease, a devastating neurodegenerative lysosomal storage disorder. The mechanism of pathogenesis underlying CLN1 disease has remained elusive....
3.
Sadhukhan T, Bagh M, Sadhukhan S, Appu A, Mondal A, Iiben J, et al.
Biochem Biophys Res Commun
. 2021 Jul;
571:137-144.
PMID: 34325129
Infantile neuronal ceroid lipofuscinosis (INCL), also known as CLN1-disease, is a devastating neurodegenerative lysosomal storage disorder (LSD), caused by inactivating mutations in the CLN1 gene. The Cln1 mice, which mimic...
4.
Sadhukhan T, Bagh M, Appu A, Mondal A, Zhang W, Liu A, et al.
J Inherit Metab Dis
. 2021 Mar;
44(4):1051-1069.
PMID: 33739454
S-palmitoylation is a reversible posttranslational modification in which a 16-carbon saturated fatty acid (generally palmitate) is attached to specific cysteine residues in polypeptides via thioester linkage. Dynamic S-palmitoylation (palmitoylation-depalmitoylation), like...
5.
Sarkar C, Sadhukhan T, Bagh M, Appu A, Chandra G, Mondal A, et al.
J Inherit Metab Dis
. 2020 Apr;
43(5):1082-1101.
PMID: 32279353
Infantile neuronal ceroid lipofuscinosis (INCL) is a devastating neurodegenerative lysosomal storage disease (LSD) caused by inactivating mutations in the CLN1 gene. CLN1 encodes palmitoyl-protein thioesterase-1 (PPT1), a lysosomal enzyme that...
6.
Appu A, Bagh M, Sadhukhan T, Mondal A, Casey S, Mukherjee A
J Inherit Metab Dis
. 2019 Apr;
42(5):944-954.
PMID: 31025705
Mutations in at least 13 different genes (called CLNs) underlie various forms of neuronal ceroid lipofuscinoses (NCLs), a group of the most common neurodegenerative lysosomal storage diseases. While inactivating mutations...
7.
Mukherjee A, Appu A, Sadhukhan T, Casey S, Mondal A, Zhang Z, et al.
Mol Neurodegener
. 2019 Jan;
14(1):4.
PMID: 30651094
Neuronal Ceroid Lipofuscinoses (NCLs), commonly known as Batten disease, constitute a group of the most prevalent neurodegenerative lysosomal storage disorders (LSDs). Mutations in at least 13 different genes (called CLNs)...
8.
Bagh M, Peng S, Chandra G, Zhang Z, Singh S, Pattabiraman N, et al.
Nat Commun
. 2017 Mar;
8:14612.
PMID: 28266544
Defective lysosomal acidification contributes to virtually all lysosomal storage disorders (LSDs) and to common neurodegenerative diseases like Alzheimer's and Parkinson's. Despite its fundamental importance, the mechanism(s) underlying this defect remains...
9.
Peng S, Xu J, Pelkey K, Chandra G, Zhang Z, Bagh M, et al.
Ann Clin Transl Neurol
. 2016 Jan;
2(12):1085-104.
PMID: 26734660
Objective: Oxidative stress in the brain is highly prevalent in many neurodegenerative disorders including lysosomal storage disorders, in which neurodegeneration is a devastating manifestation. Despite intense studies, a precise mechanism...
10.
Baker E, Levin S, Zhang Z, Mukherjee A
Ann Clin Transl Neurol
. 2015 Sep;
2(8):797-809.
PMID: 26339674
Objective: Infantile neuronal ceroid lipofuscinosis (INCL) is a devastating neurodegenerative storage disease caused by palmitoyl-protein thioesterase-1 deficiency, which impairs degradation of palmitoylated proteins (constituents of ceroid) by lysosomal hydrolases. Consequent...