Progeroid syndromes (PSs) constitute a group of disorders characterized by 
clinical features mimicking physiological aging at an early age. In some of 
these syndromes, biological hallmarks of aging are also present, whereas in 
others, a link with physiological aging, if any, remains to be elucidated. These 
syndromes are clinically and genetically heterogeneous and most of them, 
including Werner syndrome and Hutchinson-Gilford progeria, are known as 
'segmental aging syndromes', as they do not feature all aspects usually 
associated to physiological aging. However, all the characterized PSs enter in 
the field of rare monogenic disorders and several causative genes have been 
identified. These can be separated in subcategories corresponding to (i) genes 
encoding DNA repair factors, in particular, DNA helicases, and (ii) genes 
affecting the structure or post-translational maturation of lamin A, a major 
nuclear component. In addition, several animal models featuring premature aging 
have abnormal mitochondrial function or signal transduction between membrane 
receptors, nuclear regulatory proteins and mitochondria: no human pathological 
counterpart of these alterations has been found to date. In recent years, 
identification of mutations and their functional characterization have helped to 
unravel the cellular processes associated to segmental PSs. Recently, several 
studies allowed to establish a functional link between DNA repair and A-type 
lamins-associated syndromes, evidencing a relation between these syndromes, 
physiological aging and cancer. Here, we review recent data on molecular and 
cellular bases of PSs and discuss the mechanisms involved, with a special 
emphasis on lamin A-associated progeria and related disorders, for which 
therapeutic approaches have started to be developed.