NtBuHA
Mimics the depalitoylation of targets of APT1 (human-LYPLA1) or PPT1. Hydroxylamine derivative, N-(tert-Butyl) hydroxylamine (NtBuHA), is non-toxic, it cleaves thioester linkage in palmitoylated proteins and mediated lysosomal ceroid depletion in cultured cells from INCL (Infantile neuronal ceroid lipofuscinosis) patients. In Ppt1(-\/-) mice, which mimic INCL, NtBuHA crossed the blood-brain barrier, depleted lysosomal ceroid, suppressed neuronal apoptosis, slowed neurological deterioration and extended lifespan.
General
Type :
Chemical_Nomenclature : N-tert-butylhydroxylamine
Canonical SMILES : CC(C)(C)NO
InChI : InChI=1S\/C4H11NO\/c1-4(2,3)5-6\/h5-6H,1-3H3
InChIKey : XWESXZZECGOXDQ-UHFFFAOYSA-N
Other name(s) : N-tert-Butylhydroxylamine || N-(tert-Butyl)hydroxylamine || 2-Propanamine, N-hydroxy-2-methyl- || N-t-butylhydroxylamine || CHEMBL555486
MW : 89.14
Formula : C4H11NO
CAS_number : 16649-50-6
PubChem : 98586
UniChem : XWESXZZECGOXDQ-UHFFFAOYSA-N
Target
Families : No family
Protein : No protein
References (4)
| Title : APT1-Mediated Depalmitoylation Regulates Hippocampal Synaptic Plasticity - Shen_2022_J.Neurosci_42_2662 |
| Author(s) : Shen ZC , Xia ZX , Liu JM , Zheng JY , Luo YF , Yang H , Li MD , Cao T , Liu HP , Jin GL , Huang HH , Yu CX , Zhou J |
| Ref : Journal of Neuroscience , 42 :2662 , 2022 |
| Abstract : |
| PubMedSearch : Shen_2022_J.Neurosci_42_2662 |
| PubMedID: 35165175 |
| Gene_locus related to this paper: human-LYPLA1 |
| Title : GNS561, a clinical-stage PPT1 inhibitor, is efficient against hepatocellular carcinoma via modulation of lysosomal functions - Brun_2022_Autophagy_18_678 |
| Author(s) : Brun S , Bestion E , Raymond E , Bassissi F , Jilkova ZM , Mezouar S , Rachid M , Novello M , Tracz J , Hama A , Lalmanach G , Vanderlynden L , Legouffe R , Stauber J , Schubert T , Plach MG , Courcambeck J , Drouot C , Jacquemot G , Serdjebi C , Roth G , Baudoin JP , Ansaldi C , Decaens T , Halfon P |
| Ref : Autophagy , 18 :678 , 2022 |
| Abstract : |
| PubMedSearch : Brun_2022_Autophagy_18_678 |
| PubMedID: 34740311 |
| Gene_locus related to this paper: human-PPT1 |
| Title : Cln1 gene disruption in mice reveals a common pathogenic link between two of the most lethal childhood neurodegenerative lysosomal storage disorders - Chandra_2015_Hum.Mol.Genet_24_5416 |
| Author(s) : Chandra G , Bagh MB , Peng S , Saha A , Sarkar C , Moralle M , Zhang Z , Mukherjee AB |
| Ref : Hum Mol Genet , 24 :5416 , 2015 |
| Abstract : |
| PubMedSearch : Chandra_2015_Hum.Mol.Genet_24_5416 |
| PubMedID: 26160911 |
| Gene_locus related to this paper: mouse-ppt |
| Title : Neuroprotection and lifespan extension in Ppt1(-\/-) mice by NtBuHA: therapeutic implications for INCL - Sarkar_2013_Nat.Neurosci_16_1608 |
| Author(s) : Sarkar C , Chandra G , Peng S , Zhang Z , Liu A , Mukherjee AB |
| Ref : Nat Neurosci , 16 :1608 , 2013 |
| Abstract : |
| PubMedSearch : Sarkar_2013_Nat.Neurosci_16_1608 |
| PubMedID: 24056696 |
| Gene_locus related to this paper: mouse-ppt |