Cleavable crosslinkers of Formula (I) useful reagents in biochemical drug research and development such as antibody drug conjugates are disclosed. ##STR00001##

Novel substituted N-acetyl-L-cysteine (NAC) derivatives and related compounds and methods of using these compounds for the treatment of diseases and/or conditions, including but not limited to diseases and/or conditions of, or involving, the Central Nervous System (CNS), including schizophrenia adrenoleukodystrophy, mitochondrial diseases (e.g. Leigh syndrome, Alpers' disease, and MELAS), Huntington's disease, trichotillomania, HIV-associated neurocognitive disorder, hypoxic-ischemic encephalopathy, drug craving, and drug addiction.

Tiopronin prodrugs, pharmaceutical compositions comprising the tiopronin prodrugs, and methods of using tiopronin prodrugs and pharmaceutical compositions thereof for treating liver, kidney, lung, neurological, inflammatory, and autoimmune disorders including non-alcoholic steatohepatitis, Wilson's disease, cystinuria, irritable bowel disorder, ulcerative colitis, rheumatoid arthritis, chronic obstructive pulmonary disease, interstitial lung disease, asthma, cystic fibrosis, Parkinson's disease, and Huntington's disease.

Tiopronin prodrugs, pharmaceutical compositions comprising the tiopronin prodrugs, and methods of using tiopronin prodrugs and pharmaceutical compositions thereof for treating liver, kidney, lung, neurological, inflammatory, and autoimmune disorders including non-alcoholic steatohepatitis, Wilson's disease, cystinuria, irritable bowel disorder, ulcerative colitis, rheumatoid arthritis, chronic obstructive pulmonary disease, interstitial lung disease, asthma, cystic fibrosis, Parkinson's disease, and Huntington's disease.

Cysteine, N-acetylcysteine, and penicillamine related compounds, pharmaceutical compositions comprising the cysteine, N-acetylcysteine, and penicillamine related compounds, and methods of using cysteine, N-acetylcysteine, and penicillamine related compounds and pharmaceutical compositions thereof for treating liver, kidney, lung, neurological, inflammatory, and autoimmune disorders including paracetamol overdose, non-alcoholic steatohepatitis, Wilson's disease, cystinuria, irritable bowel disorder, ulcerative colitis, rheumatoid arthritis, chronic obstructive pulmonary disease, interstitial lung disease, asthma, cystic fibrosis, Parkinson's disease, and Huntington's disease.

Cysteine, N-acetylcysteine, and penicillamine related compounds, pharmaceutical compositions comprising the cysteine, N-acetylcysteine, and penicillamine related compounds, and methods of using cysteine, N-acetylcysteine, and penicillamine related compounds and pharmaceutical compositions thereof for treating liver, kidney, lung, neurological, inflammatory, and autoimmune disorders including paracetamol overdose, non-alcoholic steatohepatitis, Wilson's disease, cystinuria, irritable bowel disorder, ulcerative colitis, rheumatoid arthritis, chronic obstructive pulmonary disease, interstitial lung disease, asthma, cystic fibrosis, Parkinson's disease, and Huntington's disease.

Cysteamine prodrugs, pharmaceutical compositions comprising the cysteamine prodrugs, and methods of using cysteamine prodrugs and pharmaceutical compositions thereof for treating eye, kidney, liver, metabolic, and neurological disorders such as cystinosis, cystinuria, non-alcoholic steatohepatitis, Batten disease, Leigh syndrome, and Huntington's disease.

Cysteamine prodrugs, pharmaceutical compositions comprising the cysteamine prodrugs, and methods of using cysteamine prodrugs and pharmaceutical compositions thereof for treating eye, kidney, liver, metabolic, and neurological disorders such as cystinosis, cystinuria, non-alcoholic steatohepatitis, Batten disease, Leigh syndrome, and Huntington's disease.

Novel substituted N-acetyl-L-cysteine (NAC) derivatives and related compounds and methods of using these compounds for the treatment of diseases and/or conditions, including but not limited to diseases and/or conditions of, or involving, the Central Nervous System (CNS), including schizophrenia adrenoleukodystrophy, mitochondrial diseases (e.g. Leigh syndrome, Alpers' disease, and MELAS), Huntington's disease, trichotillomania, HIV-associated neurocognitive disorder, hypoxic-ischemic encephalopathy, drug craving, and drug addiction.

Novel substituted N-acetyl-L-cysteine (NAC) derivatives and related compounds and methods of using these compounds for the treatment of diseases and/or conditions, including but not limited to diseases and/or conditions of, or involving, the Central Nervous System (CNS), including schizophrenia adrenoleukodystrophy, mitochondrial diseases (e.g. Leigh syndrome, Alpers' disease, and MELAS), Huntington's disease, trichotillomania, HIV-associated neurocognitive disorder, hypoxic-ischemic encephalopathy, drug craving, and drug addiction.