Laminin, alpha 2

Laminin, alpha 2 is a protein that in humans is encoded by the LAMA2 gene. It is a crucial component of the basement membrane, a specialized form of extracellular matrix that provides support and anchorage for cells and tissues.

Structure

File:Laminin structure.png

Structure of Laminin, alpha 2

Laminin, alpha 2 is a large, multidomain glycoprotein that is part of the larger laminin family. It is composed of three chains: alpha, beta, and gamma, which are bound together by disulfide bonds. The alpha 2 chain, specifically, is characterized by a series of five laminin G-like (LG) domains at the C-terminus, which are involved in interactions with other extracellular matrix components and cell surface receptors.

Function

Laminin, alpha 2 plays a critical role in the formation and stability of the basement membrane. It interacts with other laminins, collagens, nidogen, and cell surface receptors to form a complex network that provides structural support to cells and tissues. In addition to its structural role, laminin, alpha 2 also influences cell behavior, including cell adhesion, migration, proliferation, and differentiation.

Clinical significance

Mutations in the LAMA2 gene can lead to a severe form of congenital muscular dystrophy known as Merosin-deficient congenital muscular dystrophy (MDC1A). This condition is characterized by muscle weakness and wasting, joint contractures, and delayed motor milestones. There is currently no cure for MDC1A, and treatment is focused on managing symptoms and improving quality of life.

See also

• Laminin
• Basement membrane
• Extracellular matrix
• Merosin-deficient congenital muscular dystrophy

External links

• LAMA2 Gene at GeneCards