Plectin
Location?
Function?
Diseases?
- located in the hemidesmosome
- links intermediate filaments to the plasma membrane, cross links hemidesmosomal proteins
- Diseases: EBS with muscular dystrophy, EBS with pyloric atresia, EBS Ogna varient (generalized contusiform bruising)
BPAG1 (BP230)
Location?
Function?
- intracellular protein in the hemidesmosomal plaque, plakin family
- binds intermediate filaments to the plaque
- plays a role in neurological disease
BPAG2 (BP180)
Location?
Function?
Diseases?
- transmembrane protein, collagen family
- Associated with BPAG1. integrin, plectin
- Diseases: BP, PG, LABD, CP (carboxy terminus), junctional EB non-Herlitz type
Integrins
Location?
Function?
Diseases?
- transmembrane protein, promotes cell-cell and cell-matrix interactions
- alpha6beta4 is in the hemidesmosomal plaque and binds intermediate filaments (intracellularly), laminin 332 and the hemidesmosomal proteins like plectin and BPAG2
- Diseases: occular CP (alpha6), JEB with pyloric atresia (beta4)
Type 7 Collagen
Location?
Function?
Diseases?
- binds the lamaina densa and either the lamina densa (to itself) or anchoring plaques
- Diseases: dystrophic EB (autosomal and dominent),bulous SLE, EBA
Laminin 332
Location?
Function?
Diseases?
- located in the lamina densa
- attaches keritinocytes to the BM
- Diseases: anti-epilegrin CP, junctional EB, Herlitz type
Cadherins
Location?
Function?
- calcium dependent cell to cell adherence molecules
- maintain tissue integrity
- includes desmogleins and desomocollins
- adhere to alpha catenin, beta catenin and plakoglobin
- classic cadherins form adherens junctions
- desmogleins/desmocollins form desmosomes
What cellular mediator is protective against urticaria
PGE2 inhibits mast cell degranulation
What amino acids are unique to elastic fibers?
Desmosine, isodesmosine
Type of secretion found in eccrine glands?
Apocrine glands?
Sebaceous glands?
Eccrine - merocrine
Apocrine - decapitation secretion
Sebaceous glands - holocrine
Which TLR recognizes lipopolysaccharide on gram-negative bacteria?
TLR4
TLR2 recognizes peptidoglycan on gram positive bacteria; associated with inflammatory acne
Which TLR recognizes viral DNA?
TLR3
TLR7 recognizes viral ssRNA; Imiquimod is a synthetic TLF7 analog
Which surface molecules do NK cells express? Which cytokines stimulate NK cells?
CD2, CD56, CD16
IL-2, IL-12, IL-15
What do mast cells release immediately upon degranulation?
Preformed mediators: Histamine Heparin (proteoglycan) Tryptase Chymase Carboxypeptidase-A Cathepsin-G
Which metal ions are necessary for proper function of matrix metalloproteinases?
Zinc (at active site) and Calcium (for structural integrity)
Cartilage is made of which collagen?
Collagen II
Which amino acids are found in elastic fibers?
Desmosine and isodesmosine
What is the function of IL-1?
Pro inflammatory: triggers innate immune response, induces fever, increases acute phase reactants
What is the function of IL-2?
Stimulates t-cells
Growth and activation of t, nk, and B cells
Function of IL-3?
Growth of mast cells
Stimulates myeloid cells
Function of IL-4 ?
Increases the TH2 response
Class switching to IgE
Function of IL-5?
Stimulates eosinophils
Stimulates B cells and IgA production
Function of IL-6?
Pro inflammatory
Stimulates B cells to differentiate to plasma cells
Increased neutrophil production
Function of IL-8?
Neutrophil chemotaxis
