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Protein Characteristics Location Function Mutations/Disorders

amyloid Misfolded proteins that Aggregation of


are nucleated instead of partially folded
degraded amyloid fibrils can
lead to Alzheimer’s.

Signal recognition Cytosol Attaches to signal sequence of


particle ER-bound peptide as it exits the
(SRP) ribosome, temporarily arrests
translation until ribosome
engages with a pore at surface of
ER.

Ubiquitin Cytosol Binds to misfolded,


dysfunctional, or unwanted
proteins and targets them for
degradation at the proteasome (3
ubiquitins are required for
degradation signal).

Chaperones ATP-dependent;attaches Cytosol and ER lumen Attempt to correct misfolded


to proteins randomly; proteins by detecting errors, such
impacted by protein- as hydrophobic residues on the
folding feedback loops surface, and giving the protein a
little squeeze.

Clathrin, COPI, Cytosol Necessary to form vesicles. First


and COPII membrane-bound cargo
receptors bind a target protein
within the lumen. Then adaptins
bind to the same cargo receptor
on the cytosolic side. Clathrin
(Golgi apparatus) or COPII (ER)
then binds to the adaptin (think of
the adaptins as a USB adaptor,
where the cargo receptor is a
USB cord and the clathrin/COPI
is a wall outlet). As more clathrin
binds to the adaptins, a bud
begins to form on the surface of
the membrane. The bud is then
pinched off by dynamin,
releasing the vesicle still coated
in clathrin (or COPII). The
scaffold then falls off, leaving a
naked vesicle transporting a
protein.

Microtubules Guide vesicular traffic Some drugs alter the


stability of
microtubules, which
impairs nerve
function.

vSNARES and vSNARES: on the There is a large variety of The organisms


tSNARES surface of vesicles. SNARES and they only interact associated with
tSNARES: on the when combined in specific pairs. tetanus and botulism
surface of membrane- This allows for targeted vesicular secrete neurotoxins
bound organelles trafficking because the vSNARE that target SNARES,
(targets of vessicles) on the vesicle will only match up thus blocking
with the proper tSNARE on the neuronal function by
target organelle. preventing vesicular
docking.

CFTR (and Two transmembrane Apical surface of Allows for passage of Cl- across Cystic Fibrosis:
ENaC) domains, two ATP- epithelial cells. cell membranes. Regulated by Decreased activity of
binding domains, a cAMP levels and phosphorylation CFTR results in a high
regulatory R domain. by protein kinase A. Related to cytoplasmic
ENaC, in that the level of Cl- in concentration of Cl-
the cell affects how much sodium and Na+ for epithelial
travels through ENaC. cells lining the airway.
Water then flows into
the cell, leaving a
thick, dehydrated
mucus.

Elastic fibers Straight fibers composed Larynx, skin, trachea, Marfan syndrome
of randomly coiled bronchi, arteries, CT
molecules. of eyes

Amorphous core protein


(elastin) surrounded by
microfibrillar glycoprotein
(fibrillin)

Contains unique amino


acids: desmosine and
isodesmosine

Fibers

Collagen Most abundant protein in Both types Type I: Resist stretch, provide Defects in collagen
body, 30% of dry weight. synthesized in RER tensile strength. synthesis can affect
More than 20 types Type I: Dermis, organ Type III: Provide structural stretch of fibers,
produced, most capsules, bone, framework and stability. strength of fibers.
commonly by fibroblasts. tendons, Excess deposition of
4 categories: fibril fibrocartilage, scar collagen in wound
forming (I (most tissue healing forms keloids.
abundant), II, III), fibril Type III (Reticular
associated, and two fibers): “extensible Triple helical structure
found in basal lamina: organs,” stroma of requires vitamin C
network forming (IV) and smooth muscle and (necessary for
anchoring fibrils (VII) hematopoietic organs, hydroxylation of
artery walls, proline residues).
intestines, uterus, Lack of vitamin C
loose connective leads to scurvy.
tissue in endocrine
glands & liver,
endoneurium

Adherin
Cadherin

Integrin

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