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View article: Niemann Pick C1 mistargeting disrupts lysosomal cholesterol homeostasis contributing to neurodegeneration in a Batten disease model
Niemann Pick C1 mistargeting disrupts lysosomal cholesterol homeostasis contributing to neurodegeneration in a Batten disease model Open
Neurodegeneration is a devastating manifestation in most lysosomal storage disorders (LSDs). Loss-of-function mutations in CLN1 , encoding palmitoyl-protein thioesterase-1 (PPT1), cause CLN1 disease, a devastating neurodegenerative LSD tha…
View article: Defective anterograde protein-trafficking contributes to endoplasmic reticulum-stress in a CLN1 disease model
Defective anterograde protein-trafficking contributes to endoplasmic reticulum-stress in a CLN1 disease model Open
Lysosomal storage disorders (LSDs) represent 70 inherited metabolic diseases, in most of which neurodegeneration is a devastating manifestation. The CLN1 disease is a fatal neurodegenerative LSD, caused by inactivating mutations in the CLN…
View article: Disruption of lysosomal nutrient sensing scaffold contributes to pathogenesis of a fatal neurodegenerative lysosomal storage disease
Disruption of lysosomal nutrient sensing scaffold contributes to pathogenesis of a fatal neurodegenerative lysosomal storage disease Open
View article: Studies on morpho-biochemical changes of longan [Euphoria longana (Lour) Steud.] fruit during fruit growth and development for determination of maturity
Studies on morpho-biochemical changes of longan [Euphoria longana (Lour) Steud.] fruit during fruit growth and development for determination of maturity Open
Longan fruit is used as fresh as well as preserved as canned due to its sweet juicy and flavoured aril and becomes available when availability of litchi is over.It is a non-climacteric fruit and continues to ripen being attached to the pla…
View article: Abstract 1502: Misrouting of Niemann-Pick C1 protein mediates cholesterol induced mTORC1-activation contributing to pathogenesis of CLN1-disease
Abstract 1502: Misrouting of Niemann-Pick C1 protein mediates cholesterol induced mTORC1-activation contributing to pathogenesis of CLN1-disease Open
View article: Abstract 1866: Loss of CLN1/PPT1 dysregulates anterograde protein-trafficking causing ER-stress and UPR in CLN1 disease
Abstract 1866: Loss of CLN1/PPT1 dysregulates anterograde protein-trafficking causing ER-stress and UPR in CLN1 disease Open
View article: Ppt1‐deficiency dysregulates lysosomal Ca<sup>++</sup> homeostasis contributing to pathogenesis in a mouse model of <scp><i>CLN1</i></scp> disease
Ppt1‐deficiency dysregulates lysosomal Ca<sup>++</sup> homeostasis contributing to pathogenesis in a mouse model of <span><i>CLN1</i></span> disease Open
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 remai…
View article: <i>Cln1</i>‐mutations suppress <scp>Rab7‐RILP</scp> interaction and impair autophagy contributing to neuropathology in a mouse model of infantile neuronal ceroid lipofuscinosis
<i>Cln1</i>‐mutations suppress <span>Rab7‐RILP</span> interaction and impair autophagy contributing to neuropathology in a mouse model of infantile neuronal ceroid lipofuscinosis Open
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 …
View article: <i>Cln3</i>‐mutations underlying juvenile neuronal ceroid lipofuscinosis cause significantly reduced levels of Palmitoyl‐protein thioesterases‐1 (Ppt1)‐protein and Ppt1‐enzyme activity in the lysosome
<i>Cln3</i>‐mutations underlying juvenile neuronal ceroid lipofuscinosis cause significantly reduced levels of Palmitoyl‐protein thioesterases‐1 (Ppt1)‐protein and Ppt1‐enzyme activity in the lysosome Open
Mutations in at least 13 different genes (called CLN s) underlie various forms of neuronal ceroid lipofuscinoses (NCLs), a group of the most common neurodegenerative lysosomal storage diseases. While inactivating mutations in the CLN1 gene…
View article: Emerging new roles of the lysosome and neuronal ceroid lipofuscinoses
Emerging new roles of the lysosome and neuronal ceroid lipofuscinoses Open
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) cause various fo…
View article: Multiple paralogues of α-SNAP in Giardia lamblia exhibit independent subcellular localization and redistribution during encystation and stress
Multiple paralogues of α-SNAP in Giardia lamblia exhibit independent subcellular localization and redistribution during encystation and stress Open
None of the genes encoding the three α-SNAPs are pseudogenes and the encoded proteins are likely to discharge non-redundant functions in the different morphological states of G. lamblia. Based on the difference in the interaction of indivi…
View article: Mannose in Complex with Colocasia esculenta Tuber Agglutinin
Mannose in Complex with Colocasia esculenta Tuber Agglutinin Open
The major tuber storage protein of Colocasia esculenta is a monocot mannose-binding, widely used, dietarylectin, for which a crystal structure was previously shown to consist of four β-prism II domains or two αβheterodimers, each forming a…
View article: Crystallization and X-ray analysis of the transcription-activator protein C1 of bacteriophage P22 in complex with the P<sub>RE</sub>promoter element
Crystallization and X-ray analysis of the transcription-activator protein C1 of bacteriophage P22 in complex with the P<sub>RE</sub>promoter element Open
The transcription-activator protein C1 of the temperate phage P22 of Salmonella typhimurium plays a key role in the lytic versus lysogenic switch of the phage. A homotetramer of 92-residue polypeptides, C1 binds to an approximate direct re…