Progeria

Progeria is a group of rare genetic disorders that cause features resembling accelerated aging, usually beginning in childhood. Hutchinson–Gilford progeria syndrome is its best-known form.

LMNA: A gene that encodes lamin A and lamin C, proteins supporting the inner structure of the cell nucleus. Most Hutchinson–Gilford cases arise from an LMNA mutation that produces abnormal progerin.
Lonafarnib: A farnesyltransferase inhibitor approved to reduce mortality risk in Hutchinson–Gilford progeria syndrome. It is the first approved drug treatment and targets a modification that helps progerin remain at the nuclear membrane.
Hutchinson–Gilford progeria syndrome: A rare disorder usually caused by an LMNA variant that leads to progerin production and childhood-onset aging-like features. This is the best-known childhood-onset form and the main focus of treatment research.
Aging: The gradual biological change over time that increases vulnerability to disease and reduces physiological resilience. Progeria offers clues about aging biology, but its mechanisms do not explain aging in full.
Progerin: An abnormal form of lamin A that remains attached to the nuclear membrane and disrupts nuclear function. This protein is the direct molecular consequence of the common Hutchinson–Gilford mutation.
Farnesyltransferase inhibitor: A drug that blocks enzymes adding farnesyl groups to proteins, altering their localization or function. Lonafarnib belongs to this drug class, whose rationale comes from progerin processing.
Werner syndrome: An autosomal recessive disorder caused by WRN variants, typically producing features of premature aging after adolescence. Its later onset distinguishes it from the most familiar childhood progeria.
Aging clock: A method for estimating biological age from measurable features such as DNA methylation patterns. Researchers examine whether progeria changes molecular age measures in ways that track disease or treatment.
Nuclear lamina: A protein meshwork lining the inner surface of the nuclear envelope and helping organize the nucleus. Progerin alters this structural layer, linking a gene defect to abnormal cell nuclei.
Base editing: A genome-editing approach that chemically changes one DNA base into another without making a double-strand break. Preclinical studies have used it to correct the disease-causing LMNA variant in models.

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Cockayne syndromeCompared with: The term highlights a shared appearance but does not capture Cockayne syndrome's repair defect or neurological course.