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13 Cards in this Set

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Where is the extra cellular matrix secreted from?


What does it contain?

Secreted from tissues + contains fibrous proteins that may be structural or adhesive or proteoglycans, these are polysaccharides linked to protein

Types of cell junctions, functions and the proteins they contain...

1)Adhesion junctions: anchor cells to one another or to the extra cellular matrix- contain cadherin proteins


2) Tight junctions: form a barrier that prevents movement of molecules from cell to cell- contain occluding proteins


3) Gap junctions: intercellular channels that allow molecules to pass from cell to cell contains connexion proteins which span two membranes, each channel is composed of 12 connexion proteins

What is connective tissue and what are the different types?

The most diverse type of tissue:


Fibrous: losse, dense, irregular and regular


Supportive: cartilage, bone


Fluid: blood lymph

What are the cells of cartilage and bone?

Chondrocytes and osteocytes: these mature cells


Chondroblasts and osteoblasts: responsible for cartilage and bone formation


Osteoclasts: responsible for breakdown and remodelling of bone



What role do membrane proteins have?

Structural


Transporters


Receptors


Enzymes

What is the cause of cystic fibrosis?


and what is the diagnosis?

Homo reccsesive CFTR gene for chlorine transport protein




Sweat test, pancreatic trypsin, idnetifactions of mutations in the DNA seqce of CTFR gene

What does the cytoskeleton contain?

Microtubules: maintenance of cell shape, cell motility, cell division, organelle movements , Actin filaments: maintenance of cell shape, motility, contraction,division


Intermediate filaments: maintain of cell shape, formation of nuclear lamina

What is the name of inherited muscle wasting disorders?


Ant the causes of this muscle wasting...

Muscle dystrophy


DMD which is most common and targets ages


1-6


BMD which is rarer and effects people 12+


both are x linked disorders


-Breakdown of a complex of cytoskeleton proteins, muscle fibres fracture and die muscle tissue is replaced by connective tissue included fat

Symptoms of CF

Respiratory problems, obstruction of gut, pancreatic problems: not enough enzymes, reduced absorbrption of food leading to malnutrition, infertility

What are lysosomal storage diseases?

Inherited diseases cause by mutations in genes encoding lysosomal enzymes or proteins responsible for delivering these enzymes to the lysosome cell, it dies when it accumulates with inactive enzymes

What is Gauchers disease, causes and symptoms?

Most common LSD, deficiency in enzyme β-glucocerebrosidase, accumulation of lips in cells


type 1= adult


type 2 = infantile


type 3 juvenile


symptoms: spleen and liver enlargement,distension of the colon,recurrent pulmonary infections,recurrent pulmonary infections,anaemia,pain in body, limbs and joints,neurological abnormalities

Another type of LSD symptoms, causes....

Inclusion cell disease: recessive, deficiency of a phosphotrahsferase that is involved in directing proteins to the lysosome


symptoms: poor development of bone, respiratory problems, hernias, respiratory insufficiency

What are protofilaments made from? And what can these form?

Composed of the proteins, α- and β-tubulin, αβ-heterodimers join end-on-end to form a protofilament


13 protofilaments assemble to form a hollow cylinder with a diameter of ~25 nm