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MLS 2213 UNIT 11; HEME DERIVATES TEST

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MLS 2213 UNIT 11; HEME DERIVATES TEST...

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  • October 22, 2024
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  • MLS 2213 UNIT 11; HEME DERIVATES
  • MLS 2213 UNIT 11; HEME DERIVATES
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MLS 2213 UNIT 11; HEME DERIVATES TEST

Binds to iron and is involved in iron transport throughout the body
Transferrin
Describe the difference between heme and haemoglobin
Heme: protoporphyrin ring + Fe

Hemoglobin: Heme + 4 globulin chains
Common globulin chains
alpha (𝞪), beta (𝛃), delta (𝛅), and gamma (𝞬)
Site of iron absorption
Small intestine
Iron in what form is toxic to the tissues?
Unbound
Ferritin: Function? Iron form? Location?
Function: storage form of iron

Form: Ferric form (3+)

Location: Liver
Functional form of iron primarily in the blood
Ferrous (2+)
Porphyrins
Class of pigments whose molecules contain a flat ring of four linked
heterocyclic groups (possibly with a central mental ion)
Porphyrias
(purple in greek)

Diseases caused by enzyme deficiencies in the heme production
pathway
A black inorganic compound formed from heme when RBCs are lysed
and exposed to air. (Can be a treatment for some porphyrias)
Hemin
Primary Porphyrias
Porphyria Cutanea Tarda, Acute Intermittent Porphyria, Erythropoietic
Porphyria, ALA-Dehydratase Deficiency
Porphyria Cutanea Tarda: Deficiency? Best test to order? Defined by?

, Deficiency: Uroporphyrinogen Decarboxylase

Best test to order: Serum porphyrin

Defined by: Blistering
Acute Intermittent Porphyria: Deficiency? Best test to order? Defined by?
Deficiency: PBG Deaminase

Best test to order: urine porphobilinogen

Defined by: blistering and nerve impairments
Erythropoietic Porphyria: Deficiency? Increase in? Defined by?
Deficiency: Ferrochelatase

Increase in: total erythrocyte protoporphyrin

Defined by: blistering
Enzyme deficiency affecting the rate-limiting step in porphyrin synthesis
ALA-Dehydratase Deficiency
ALA-Dehydratase Deficiency: Results in? Confounded by? Presenting
symptom?
Results in: accumulation of delta-aminolevulinic acid

Confounded by: lead poisoning

Presenting symptom: abdominal pain
Secondary Porphyrias
Lead poisoning, hereditary tyrosinemia, liver disease, iron deficiency
anemia
Inhibits most enzymes in the synthetic pathway
Lead poisoning
Causes elevated levels of succinylacetone leading to elevated levels of
d-Aminolevulinic Acid
Hereditary tyrosinemia
Causes a problem synthesizing heme
liver disease
Leads to an increase in heme production and Erythrocyte protoporphyrin
Iron Deficiency Anemia

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