What is the major source and function of  Chylomicrons?

Source: intestine

Function: transport of dietary TG's

Source: Liver

Function: transport of endogenously synthesized TG's

Source: formed in circulation by partially broken down IDL

Function: delivers cholesterol to peripheral tissues

Source: Liver

Function: Removes "used" cholesterol from tissues and takes it to the liver, also donates apolipoproteins to chylomicrons and VLDL

• A
• B48
• C (I, II, III)
• E

• B100
• C (I,II,III)
• E

• B100

• A
• C(I,II,II)
• D
• E

What is the major importance/function of Apo-B48?

• Carries cholesterol esters
• lacks LDL receptor binding domain 
• only found in chylomicrons

Increased Production:

Increased precursors or Increased insulin

Decreased Removal:

Diabetes, Renal Disease, Lipoprotein and Enzyme abnormalities 

• Total Chol. LDL, Trigs, HDL
• Diabetes, hypertension
• Obesity, smoking, inflammatory markers
• medical history, physical condition

1. LDL/Lipoprotein reminants are deposited into the subendothelial space, then gets taken up by various cells (like macrophages---foam cells form)
2. this promotes inflammatory response (plaque starts to form)
3. injury signals promote expression of adhesion molecules and cell proliferation of macrophages, lymphs, and platelets to the plaque
4. Continual injury and repair leads to additional narrowing of the vessel
5. Final event leading to thrombus that blocks blood flow (complete occlusion) is hemorrhage into the plaque

• HDL: >60 mg/dL
• LDL: <130 mg/dL
• Trigs: <200 mg/dL
• Total Cholesterol: <200 mg/dL

Cause: Deficient LPL activity

Deficient or defective apo-CII 

Abnormalities: Increased Chylomicrons, Increased Trigs, Pancreatitis, DM.

Cause: Defects in expression or function of LDL receptor

LDL receptor gene def. 

Abnormalities: Increased LDL Increased TC

Cause: High Apo-B100 synthesis

Abnormalities: Increased VLDL, TC, LDL, and Trigs

Cause: Abnormal IDL metabolism, defect VLDL remnant clearing, defective Apo-E

Abnormalities: Increased IDL, TC, Trigs, "Broad Beta"

Cause: Abnormal VLDL metabolism

Abnormalities: Increased Trigs, VLDL, Hyperuricemia

Cause: Abnormal VLDL / Chylomicron metabolism, Defective Lipolysis

Abnormalities: Increased Trigs, VLDL, Chylomicrons

Def. in Apo-CII is seen in type 1 hyperlipidemia but may show as what other type?

1. Type IIb Fam combinined Hyperlipidemia
2. Hyperapobetalipoproteinemia
3. Type IV Fam Hypertriglyceridemia 
4. Type V Hyperlipoproteinemia
5. Type II Dysbetalipoproteinemia

Fam. Alphalipoproteinemia

Defects in synthesis of Apo-A1

Defects in catabolism of Apo-A1

--Endocrine/Metab: diabetes, hypothyroidism, cushings, addisons, glycogen storage, porphyria

--Renal Dis: Uremia, Nephrotic syndrome, multiple myeloma

--Hepatic: Prim. biliary cirrhosis, Acute hepatitis, Pancreatitis

--Peripheral: Gout, Pernicous Anemia

Thiazides 

Glucocorticoids

Anabolid Steroids

Beta Blockers 

Oral Concentraceptive 

ETOH (excessive intake)

What Classification would this be: 

Total Chol: 300

Trig: 180

LDL: 264

Peripheral Arterial Disease

Abdominal Aortic Aneurysm

Carotid Artery Disease

Lp(a)

-Competes with plasminogen for fibrin receptors increasing plaque formation

-promotes thrombosis and cell adhesion

Hyperalphalipoproteinemia 

Cholesterol Ester Transfer Protein Def.

High ethanol intake 

Excerise 

Drugs (estrogens, fibrates, nicotnic acid, statins)

Fam. Hyplipoproteinemia

Apo A1 abnormality

Tangiers Dis

LCAT def.

Smoking 

obestiry 

metabolic syndrome 

CKD

Drugs (testosterone, progesterone, steroids)

LCAT continues at RT 

Trigs/Glycerol concentrations increase at RT

used for apolipoprotein measurement

TC, TG, HDL multiply by 1.03

Age:increases with age

Gender: no difference until the age of 35 men increases faster until the age of 55 (post menopausal increases at a greater rate)

What are the lipid storage diseases?

Guachers 

Niemann pick

Tay-sachs (GM2)

Fabrys 

GMIgangliosis