Lipids and Proteins Flashcards
When should chylomicrons be found in plasma?
VLDL are major carrier of what?
IDL’s carry?
LDLs?
HDLs?
Post prandial
VLDL: Tgs
IDL: Tgs and cholesterol
LDL: Cholesterol
HDL: REVERSE cholesterol carrier
Where are chylo’s made (exogenous/ feeding)?
LPL cleaves chylo’s to?
Then they go where?
Endogenous LDL pathway (fasting)?
What ends up causing foam cells?
Intestine then LPL (lipoprotein lipase)–> chylomicron remnants–> liver LDL-R
Free fatty acid and monoglycerated acids
Endo: LIver–> VLDL–LPL–>IDL—–Liver LDL-R
or IDL–Hepatic Lipase–>LDL—-LDL-R; some LDL gets oxidized and needs scavenger receptors
NON-HDL cholesterol measurement equation?
What abetalipproteninemia is in intestines, liver?
Really low VLDL and LDL can be a sign of?
Risk for atherosclerosis (LDL-C + IDL-C + VLDL-C); can be measured non-fasting
Apo B 48, Apo B 100
Liver disease
Increased’s are increases in some or all of what types of molecules?
Increased LDL’s increase in?
In crease in Tg and LDL-C?
Tg: Chylo’s (HLP I; Rare), VLDLs (HLP IV, common), or both (V)
LDL-c: LDL (IIA, common)
LDL-C and Tg: LDL and VLDLs (IIB/ common), IDL and remnant LP’s (III, Rare)
Presentation of hyperchyloiconemia?
3 types (hint what carries and breaks down chylo’s)?
Tgs>1000
LPL deficiecney; Auto recessive, 1e6; Infant onset
Apo CII deficiency: Ligand for LPL, Auto recessive; child onset
SLE: Acuired; Auto ab to LPL
Increased VLDLs caused by (Genetic; type IV))?
Aquired?
Insulin resistance (receptor mutation or lipodystrophy); Familal hyertriglyceridemia (Apo B increase)
Inborn errors
Acquired: Many; Insulin resistance, Obesity, Disbetes, cushings, Pheo, steroid, liver/renal disease, and MORE!
Causes of high LDL (Mono Genetic)?
Mongenic: Familial hypercholsteroma (**LDL receptor defect)**, **Famialiay hyperapo-B-lipoproteinemia (LDL receptor ligand)**
Famalial combined hyperlipidemia
AR familial hypercholesteromia
PCSK9 gain of function (LDL receptor protein that brings it into cell from surface)
High LDL (acquired)?
Liver disease, renal disease, hypothyroidism, Diabetes, glucorticoids and androgens
Familail hyerpap-B-lipproteinemia has what increased?
Rare type III increased IDL and rem LP and causes?
LDL but not VLDL
Apo E2/E2 homozygous; caused by Ethanol, DM, Renal, Liver
What is abetalipoproteinemia red cell finding?
Labs, Tg, Chol, VLDL, LDL, HDL?
What age does it present?
Acantocytes (non sym. projections)
Tg, Chol, LDL, VLDL low; HDL perserved
In infants as failure to thrive
What IL drives acute phase reactants?
Some positive acute phase reactants?
Negative acute phase reactants (down in concentration)?
IL-6
C3, CRP, A1AT, A2M, Ceruloplasmin, Fibrinogen, VIIII, VwF, Haptoglobin
Negative: Albumin, Transhtyretin (pre-albumin/nutritional marker), Retinol binding protein, transferrin
What causes decrease in total protein?
What causes increase in total protein?
Hypolabuminemia, hypogamma., panhypoprotenemia
Increased: Dehydration, poly/mono gammopathy, prolonged tourniquet time
On protein gel what runs in pre-albumin ?
Pre-Albumin: Retinol-binding protein (vit A transporter) and Transthyretin (T4 transport); Maybe seen in CSF
Alpha - 1 region proteins/functions?
Decreased in?
A1AT- protease inhibitor
HDL- Reverse cholesterol transport
TBG- T3, T4 transport
CBG (transcortin): cortisol
Orosomucoprotein. Alpha-1-glycoprotein: immune response modifier
Prothrombin: plasma protein
Decreased: A1AT def. HDL defic
Inc: Acute inflammation
A1AT deficiency casues liver damage by?
Lung damage from?
Protein name?
Genotypes?
Failure to export protein (unless null)
Inability to inhibit elastase
- *SERPIN1**
- *MM: normal**
MZ: asymptomatic
ZZ: disease
Alpha 2 region proteins/functions?
Increase?
Decrease?
Alpha 2 macroglobulin-protease inhibitor
Haptaglobin-Hb binding; down with hemolysis; some blacks lack it but no clinical consequence; hemapexin binds free heme
Ceruloplasmin-Oxidation-reduction reaction –Fe2+–> Fe3+++
Inc: Acute inflammation, nephrosis (A2M increase)
Beta band proteins/functions?
C3 deficency causes and C5-9 deficency?
Increases in?
Transferrin-Fe transport
Hemopexin-Heme binding (blacks with ahaptaglobinemia)
LDL- Chol transport
C3: Complement cascade; deficency=encapsulated organisms H influ. Staph and strep; terminal complements C5-9=meningococcal infection
Inc: Liver disease (Beta-Gamma bridge), hyperlipidiemia, Nonfasting
What proteins run near the Beta-gamma interface?
IgA
Fibrinogen–common artifact!
What runs in the gamma area?
What to think about if low gamma and high alpha 2 and disease kids get?
What classic infection does galactosemia have?
IgM- primary humoral response
IgG: 1st and 2nd adaptive response: crosses placenta
CRP: Inate immunity and initiates classic complement pathway
Think renal disease!; kids get peritoneal pneumococcal infection
E. Coli
MGUS definition by spike and BM plasma cells?
MM risk?
Monoclonal gammopathy that does not meet MM diagnosis or neoplastic casue of mono gammopathy
Asymptomatic
M-spike <3 g/dL; BM plasma <10%
Risk of MM: 1%/yr
What is Waldenstroms macroglobulinemia?
Are there bone lesions?
Is the hyperviscosity better or worse than MM?
IgM (pentamer) monoclonal
Lymphoma like/Lymphoplasmacytic lymphoma
No bone lesions
Hyperviscosity is worse
What neoplastic plasma cell dyscrasia produce mono gammopathies?
MM/plasmacytoma
Monoclonal immunoglobulin depositoin disease
Osteosclerotic myeloma
AL amyloidosis (Beta-sheets); deposits in tissues eg in tongue, hepatomegaly, glomerular disease, GI tract issues, etc.
MGUS vs smoldering myeloma, MM (treated):
M-spike?
BM%
Symptoms?
CRAB?
MGUS: M-spike <3 g/dL AND BM <10% plasma; no symptoms
Smoldering: > or equal to 3 g/dL OR 10% or more BM Plasma cells; no symptoms
M-spike maybe present, BM usually >10% plasma cells, Yes to 1 MRI lesion or Abn light chain ratio or BM plasma cells >60%
Hypercalcimia, Renal disease, Anemia, Bone pain/lesions
Causes of hypgammaglobuninemia (primary)?
Seconday?
Brutons agammaglobuninemia; X-linked; BTK gene defect–B-precell signaling and causes BTK stopping at Pre-B Cell
CVID
IgA deficency
Hyper-IgM syndrome (CD40L XLR disorder, CD40, NEMO, AID, UNG)
Secondary: Loss, immunosupression, chemo, irradiation, Immuno for organ donation, light chain disease (monoclonal light chain negative feedbacks)
