ExtracellularIsoformsDystroglycanIntracellularBasement membraneLAMB1ProteinsInteractionsTissuesDeficiencyProteinAxonsAdhesionAlphaAdditionSpecificMDC1AMerosinAutosomal recessiveExtracellular matrixHeterotrimericMuscular dystrophyIntegrin receptorsNomenclatureFibronectinMyofibersPeripheral nerveOccursPhenotypeMoleculesStructuralChainsMajorFamilyPresenceRange
Extracellular3
- Laminins are a family of glycoproteins of the extracellular matrix of all animals. (wikipedia.org)
- Laminins are integral to the structural scaffolding of almost every tissue of an organism-secreted and incorporated into cell-associated extracellular matrices. (wikipedia.org)
- Laminins are extracellular matrix proteins that reside in the basement membrane and provide structural support in addition to promoting cellular adhesion and migration. (bvsalud.org)
Isoforms2
- In metabolic tissues such as the pancreas, adipose, muscle, and liver, laminin isoforms are expressed in discrete temporal and spatial patterns suggesting that certain isoforms may support the development and function of particular metabolic cell types. (bvsalud.org)
- This review focuses on the research to date detailing the expression of laminin isoforms, their potential function, as well as known pathways involved in laminin signaling in metabolic tissues. (bvsalud.org)
Dystroglycan1
- The proteins also bind to cell membranes through integrins and other plasma membrane molecules, such as the dystroglycan glycoprotein complex and Lutheran blood group glycoprotein. (wikipedia.org)
Intracellular2
- citation needed] This effect of laminin-111 probably occurs through a lowering of intracellular cyclic AMP. (wikipedia.org)
- Through interactions with cell surface receptors, laminins stimulate intracellular signaling cascades which direct specific survival and differentiation outcomes. (bvsalud.org)
Basement membrane1
- Abnormal laminin-332, which is essential for epithelial cell adhesion to the basement membrane, leads to a condition called junctional epidermolysis bullosa, characterized by generalized blisters, exuberant granulation tissue of skin and mucosa, and pitted teeth. (wikipedia.org)
LAMB11
- Laminin subunit beta 1 (LAMB1) is involved in attachment, migration, and organization during development, and its elevated expression has been associated with several cancers. (bvsalud.org)
Proteins1
- Laminins are heterotrimeric proteins with a high molecular mass (~400 to ~900 kDa) and possess three different chains (α, β and γ) encoded by five, four, and three paralogous genes in humans, respectively. (wikipedia.org)
Interactions1
- Through these interactions, laminins critically contribute to cell attachment and differentiation, cell shape and movement, maintenance of tissue phenotype, and promotion of tissue survival. (wikipedia.org)
Tissues1
- We will also discuss the current biomedical therapies involving laminins in these tissues in addition to prospective applications, with the goal being to encourage future investigation of laminins in the context of metabolic disease. (bvsalud.org)
Deficiency1
- Cardiac complications are especially common in patients with a mutation in FKRP and occasionally in patients with laminin-α2 deficiency. (medscape.com)
Protein1
- Malfunctional laminin-521 in the kidney filter causes leakage of protein into the urine and nephrotic syndrome. (wikipedia.org)
Axons1
- Laminin-111 is a major substrate along which nerve axons will grow, both in vivo and in vitro. (wikipedia.org)
Adhesion1
- For example, the peptide sequence [GTFALRGDNGDNGQ], which is located on the alpha-chain of laminin, promotes the adhesion of endothelial cells. (wikipedia.org)
Alpha5
- The laminins are combinations of different alpha-, beta-, and gamma-chains. (wikipedia.org)
- Description: Quantitative sandwich ELISA for measuring Rat Leucine-rich alpha-2 glycoprotein 1 (LRG1) in samples from cell culture supernatants, serum, whole blood, plasma and other biological fluids. (resourcerecovery.be)
- Description: Quantitativecompetitive ELISA kit for measuring Human leucine-rich alpha-2 glycoprotein 1 (LRG1) in samples from serum, urine, cell culture supernates, tissue homogenates. (resourcerecovery.be)
- Description: A competitive ELISA for quantitative measurement of Rat Leucine rich Alpha 2 glycoprotein 1 in samples from blood, plasma, serum, cell culture supernatant and other biological fluids. (resourcerecovery.be)
- Description: Quantitative sandwich ELISA for measuring Human Leucine-rich alpha-2 glycoprotein 1 (LRG1) in samples from cell culture supernatants, serum, whole blood, plasma and other biological fluids. (resourcerecovery.be)
Addition1
- In addition, many laminins had common names before either laminin nomenclature was in place. (wikipedia.org)
Specific1
- Some of these biological functions of laminin have been associated with specific amino-acid sequences or fragments of laminin. (wikipedia.org)
MDC1A5
- MDC1A is caused by mutations in the laminin α2 gene ( LAMA2 ) linked to chromosome 6q22-q23 and inherited as autosomal recessive. (medscape.com)
- Clinical variants of MDC1A occur with some mutations when only partial laminin-α2 deficiency is present. (medscape.com)
- Congenital muscular dystrophy (CMD) type 1A (MDC1A) is caused by recessive mutations in laminin-α2 (LAMA2) gene. (nih.gov)
- The clinical manifestations of LAMA2 muscular dystrophy ( LAMA2 -MD) comprise a continuous spectrum ranging from severe congenital muscular dystrophy type 1A (MDC1A) to milder late-onset LAMA2 -MD. MDC1A is typically characterized by neonatal profound hypotonia, poor spontaneous movements, and respiratory failure. (nih.gov)
- The phenotypic spectrum of LAMA2 muscular dystrophy ( LAMA2 -MD) ranges from congenital muscular dystrophy type 1A (MDC1A) to LAMA2 -MD with onset ranging from early childhood to adulthood (referred to as late-onset LAMA2 -MD). (nih.gov)
Merosin2
- The major laminin of adult skeletal muscle is laminin-2 (also known as merosin), and only mutations of LAMA2 gene encoding laminin α2 cause muscular dystrophy. (medscape.com)
- A large series of patients with LAMA2 mutations was described, highlighting the differences between the severe homogenous presentation typical of patients with absent merosinimmunostaining with the more heterogeneous presentation of those with residual merosin expression. (medscape.com)
Autosomal recessive1
- LAMA2 -MD is inherited in an autosomal recessive manner. (nih.gov)
Extracellular matrix4
- Laminins are a family of glycoproteins of the extracellular matrix of all animals. (wikipedia.org)
- Laminin-211, a heterotrimeric glycoprotein that contains the α2 chain, is crucial for muscle stability establishing a bond between the sarcolemma and the extracellular matrix. (nih.gov)
- Alpha-dystroglycan is an extracellular peripheral glycoprotein that acts as a receptor for both extracellular matrix proteins containing laminin-G domains. (nih.gov)
- Secreted molecules also contribute to the extracellular matrix (fibronectin, laminin, reelin). (frontiersin.org)
Heterotrimeric1
- Laminins are heterotrimeric proteins with a high molecular mass (~400 to ~900 kDa) and possess three different chains (α, β and γ) encoded by five, four, and three paralogous genes in humans, respectively. (wikipedia.org)
Muscular dystrophy3
- defective laminins can cause muscles to form improperly, leading to a form of muscular dystrophy, lethal skin blistering disease (junctional epidermolysis bullosa), and/or defects of the kidney filter (nephrotic syndrome). (wikipedia.org)
- Dysfunctional structure of one particular laminin, laminin-211, is the cause of one form of congenital muscular dystrophy. (wikipedia.org)
- The diagnosis of LAMA2 muscular dystrophy is established in a proband with suggestive findings and biallelic ( homozygous or compound heterozygous ) pathogenic variants in LAMA2 identified by molecular genetic testing . (nih.gov)
Integrin receptors2
- Neurons of the peripheral nervous system express integrin receptors that attach to laminins and promote neuroregeneration after injury. (wikipedia.org)
- Osteopontin is a secreted matricellular glycoprotein that signals partially through integrin receptors and its downregulation is associated with increased strength, reduced fibrosis, and milder muscle pathology 10 , 11 . (nature.com)
Nomenclature1
- In addition, many laminins had common names before either laminin nomenclature was in place. (wikipedia.org)
Fibronectin2
- Laminins form independent networks and are associated with type IV collagen networks via entactin, fibronectin, and perlecan. (wikipedia.org)
- For example, growth cones are repelled by netrin when grown on laminin-111 but are attracted to netrin when grown on fibronectin. (wikipedia.org)
Myofibers1
- Laminins are extracellular glycoproteins that bind with other extracellular and transmembrane proteins to form the frame of the basal lamina that surrounds individual myofibers. (medscape.com)
Peripheral nerve2
- citation needed] Laminins are enriched at the lesion site after peripheral nerve injury and are secreted by Schwann cells. (wikipedia.org)
- Receptor for laminin-2 (LAMA2) and agrin in peripheral nerve Schwann cells. (nih.gov)
Occurs1
- citation needed] This effect of laminin-111 probably occurs through a lowering of intracellular cyclic AMP. (wikipedia.org)
Phenotype1
- Through these interactions, laminins critically contribute to cell attachment and differentiation, cell shape and movement, maintenance of tissue phenotype, and promotion of tissue survival. (wikipedia.org)
Molecules2
- The laminin molecules are named according to their chain composition, e.g. laminin-511 contains α5, β1, and γ1 chains. (wikipedia.org)
- The three short arms have an affinity for binding to other laminin molecules, conducing sheet formation. (wikipedia.org)
Structural1
- Laminins are integral to the structural scaffolding of almost every tissue of an organism-secreted and incorporated into cell-associated extracellular matrices. (wikipedia.org)
Chains3
- Each laminin is a heterodimer composed of a heavy chain (α) and two light chains (β and γ). (medscape.com)
- The laminins are combinations of different alpha-, beta-, and gamma-chains. (wikipedia.org)
- Laminin-211 is composed of an α2, β1 and γ1 chains. (wikipedia.org)
Major1
- Laminin-111 is a major substrate along which nerve axons will grow, both in vivo and in vitro. (wikipedia.org)
Family1
- Once the LAMA2 pathogenic variants have been identified in an affected family member, carrier testing for at-risk relatives and prenatal and preimplantation genetic testing are possible. (nih.gov)
Presence2
- White-matter changes are not correlated with the amount of laminin-α2, the patient's intelligence, or the presence of seizures. (medscape.com)
- The presence of laminin-1 can influence how the growth cone responds to other cues. (wikipedia.org)
Range1
- In late-onset LAMA2 -MD onset of manifestations range from early childhood to adulthood. (nih.gov)