The names 'acquired junctional epidermolysis bullosa', 'anti-laminin-332 mucous membrane pemphigoid (MMP)' and 'mixed auto-immune subepidermal blistering dermatosis' are proposed for dogs with clinical signs reminiscent of EBA, MMP or BP respectively. (morulaivf.com)
An increasing number of studies has highlighted the association of anti-laminin 332 MMP and malignancies that can be revealed in about a quarter of these patients. (bvsalud.org)
This data has led to the recommendation of current guidelines to assay for anti-laminin 332 reactivity in all MMP patients. (bvsalud.org)
The present review focuses on anti-laminin 332 MMP describing clinical features, its pathophysiology, and detection of serum anti-laminin 332 IgG. (bvsalud.org)
In addition, the available data about the occurrence of malignancies in anti-laminin 332 MMP, the underlying tumor entities, and its biology are detailed. (bvsalud.org)
Typical clinical and immunopathological characteristics of MMP, such as oral, conjunctival and skin lesions, are reflected, for example, in an antibody transfer-induced mouse model for anti-laminin 332 MMP in adult mice. (bvsalud.org)
Junctional epiderm1
The importance of laminin 332 for the structural integrity of the BMZ is demonstrated by mutations in any of the three genes encoding for its three chains causing variants of junctional epidermolysis bullosa. (bvsalud.org)
Protein6
The protein encoded by this gene belongs to the laminin family of secreted molecules. (utsouthwestern.edu)
Interleukin-2 receptor (IL-2R) is a heterotrimeric protein expressed on the surface of certain immune cells, such as lymphocytes, that binds and responds to the cytokine IL-2. (transcriptionfactor.org)
Analyzing the protein expression patterns of Fas, FADD, caspase 8, CDC25B, fascin, CK14, CK4, annexin I, laminin-5γ2 and SPARC would be valuable to develop rational strategies for early detection of lesions at risk in advance as well as for prevention and treatment of ESCC. (biomedcentral.com)
Mucous membrane p1
Autoimmunity against laminin 332 is observed in mucous membrane pemphigoid (MMP) and in the rare patients with orf-induced pemphigoid. (bvsalud.org)
Subunit1
see HBG2 Gamma secretase Laminin, gamma 1 Laminin, gamma 2 This disambiguation page lists articles associated with the title Gamma subunit. (wikipedia.org)
Alpha31
It is a heterotrimeric molecule composed of alpha1(V), alpha2(V) and alpha3(V) subunits. (lookformedical.com)
Collagen3
Immunological investigations consisted of direct immunofluorescence (IF), indirect IF with intact and salt-split canine gingival, and salt-split normal or laminin-332-deficient human skin, immunoblotting with purified human laminin-332 and immunoblotting with recombinant NC1 domain of human collagen VII. (morulaivf.com)
Type IV collagen is the major structural component of glomerular basement membranes (GBM), forming a 'chicken-wire' meshwork together with laminins, proteoglycans and entactin/nidogen. (cusabio.com)
The main autoantigens of MMP are type XVII collagen (BP180) and laminin 332 targeted in about 80% and 10-20% of patients, respectively. (bvsalud.org)
In five dogs, IgG variably recognized the basement membrane of laminin-332-deficient human skin (three dogs negative, two dogs positive). (morulaivf.com)
These observations establish laminin-332 as a novel basement membrane antigen in dogs with autoimmune blistering diseases with variable clinical phenotypes. (morulaivf.com)
Proteins1
To confirm the immunohistochemical results, three proteins, fascin, CK14 and laminin-5γ2, which were overexpressed in ESCC on tissue microarray, were detected in 12 ESCC cell lines by Western blot assay. (biomedcentral.com)
Domain1
Laminins are heterotrimeric molecules that consist of alpha, beta, and gamma subunits that assemble through a coiled-coil domain. (utsouthwestern.edu)
Immune1
One major pathogenic pathway of skin blistering and inflammation is activation of myeloid cells following Fc gamma receptor-dependent binding to the skin-bound immune complexes. (bvsalud.org)
Glycoproteins4
In particular, the laminins, a family of heterotrimeric extracellular glycoproteins, affect tissue development and integrity in such diverse organs as the kidney, lung, skin, and nervous system. (wikipedia.org)
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)
Subunits2
Laminins are heterotrimeric molecules that consist of alpha, beta, and gamma subunits that assemble through a coiled-coil domain. (utsouthwestern.edu)
Laminins function as heterotrimeric complexes of alpha, beta, and gamma chains, with each chain type representing a different subfamily of proteins. (wikipedia.org)
The protein encoded by this gene belongs to the alpha subfamily of laminin chains and is a major component of basement membranes. (wikipedia.org)
Similar to all members of the family of lamininproteins, laminin-332 is a large heterotrimeric molecule containing alpha-3, beta-3, and gamma-2 chains. (medscape.com)
Laminins, composed of 3 non identical chains: laminin alpha, beta and gamma (formerly A, B1, and B2, respectively), have a cruciform structure consisting of 3 short arms, each formed by a different chain, and a long arm composed of all 3 chains. (nih.gov)
The gamma 2 chain together with alpha 3 and beta 3 chains constitute laminin 5 (earlier known as kalinin), which is an integral part of the anchoring filaments that connect epithelial cells to the underlying basement membrane. (nih.gov)
Epithelial cell migration during wound healing is regulated in part by enzymatic processing of laminin-332 (formerly LN-5), a heterodimer formed from alpha, beta, and gamma polypeptide chains. (nih.gov)
During incisional wounding, laminin gamma2 chains in particular are processed to smaller sizes and function to promote epithelial sheet migration over the wound bed. (nih.gov)
SM exposure greatly increased mRNA levels for laminin-gamma2 in comparison to the other two chains. (nih.gov)
Each laminin is a heterodimer composed of a heavy chain (α) and two light chains (β and γ). (medscape.com)
Molecules1
In addition, anchoring filaments contain the molecules laminin-332 and laminin-6. (medscape.com)
Embryonic4
It is thought that laminins mediate the attachment, migration, and organization of cells into tissues during embryonic development by interacting with other extracellular matrix components. (wikipedia.org)
Binding to cells via a high affinity receptor, laminin is thought to mediate the attachment, migration and organization of cells into tissues during embryonic development by interacting with other extracellular matrix components. (nih.gov)
Polyclonal and monoclonal antibodies against chicken gizzard 5 '-nucleotidase inhibit the spreading process of chicken embryonic fibroblasts on laminin substratum. (morulaivf.com)
Polyclonal and monoclonal antibodies raised against chicken gizzard 5′-nucleotidase were tested in adhesion assays of embryonic chicken fibroblasts (CEF) for their ability to interfere with the adhesion process of these cells on either laminin or fibronectin substrata. (morulaivf.com)
Epithelial3
It is expressed in several fetal tissues but differently from gamma 1, and is specifically localized to epithelial cells in skin, lung and kidney. (nih.gov)
The mouse ear vesicant model (MEVM) was used to follow the pathology in the ear and test whether processed laminin-332 enhances epithelial cell migration. (nih.gov)
Using the same approach, human primary or PSC-derived BCs transplanted into NOD scid gamma (NSG) recipient mice similarly display multilineage airway epithelial differentiation in vivo. (bvsalud.org)
In five dogs, IgG variably recognized the basement membrane of laminin-332-deficient human skin (three dogs negative, two dogs positive). (morulaivf.com)
These observations establish laminin-332 as a novel basement membrane antigen in dogs with autoimmune blistering diseases with variable clinical phenotypes. (morulaivf.com)
Muscular1
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)
Attachment2
The epithelium-specific expression of the gamma 2 chain implied its role as an epithelium attachment molecule, and mutations in this gene have been associated with junctional epidermolysis bullosa, a skin disease characterized by blisters due to disruption of the epidermal-dermal junction. (nih.gov)
The initial attachment process of CEF on fibronectin and laminin substrata was not influenced by preincubating these cells with antibodies against chicken gizzard 5′-nucleotidase. (morulaivf.com)
Presence2
White-matter changes are not correlated with the amount of laminin-α2, the patient's intelligence, or the presence of seizures. (medscape.com)
Clues to the presence of laminin-α2 deficiency include MRI abnormalities, seizures, and demyelinating neuropathy. (medscape.com)
Alpha2
Laminin, alpha 5 has been shown to interact with BCAM. (wikipedia.org)
Different alpha, beta and gamma chain isomers combine to give rise to different heterotrimeric laminin isoforms which are designated by Arabic numerals in the order of their discovery, i.e. alpha1beta1gamma1 heterotrimer is laminin 1. (nih.gov)
Chain2
This gene encodes the gamma chain isoform laminin, gamma 2. (nih.gov)
Two transcript variants resulting from alternative splicing of the 3' terminal exon, and encoding different isoforms of gamma 2 chain, have been described. (nih.gov)
Human2
Laminin-332 polypeptides are targeted by auto-antibodies in human patients with mucous membrane (cicatricial) pemphigoid or, more rarely, subepidermal vesicular diseases that resemble epidermolysis bullosa acquisita (EBA) or bullous pemphigoid (BP). (morulaivf.com)
In all nine dogs, IgG auto-antibodies detected purified human laminin-332 by immunoblotting. (morulaivf.com)
Cells2
However, the subsequent spreading process of these cells was found to be inhibited for at least 2 h on a laminin substratum. (morulaivf.com)
Spreading-arrested and rounded CEF do not develop prominent intracellular stress-fibers like control cells, instead they seem to concentrate their available actin in areas of presumptive initial contact with the laminin substratum. (morulaivf.com)
Mouse1
Upregulation of gamma-2 laminin-332 in the mouse ear vesicant wound model. (nih.gov)
Present1
Clinical variants of MDC1A occur with some mutations when only partial laminin-α2 deficiency is present. (medscape.com)