MINERAL

METABOLISM YASAR ARAFAT

ROLL NO : 24

- Copper (Cu) -----

TAHA MUQRI
- Manganese (Mn) ROLL NO : 75
-----
VIGNESH KUMAR
ROLL NO : 137
  Total body copper is about 100 mg.
COPPER (Cu)  It is seen in muscles, liver, bone marrow, brain, kidney, heart and in
hair.
Copper is an essential constituent of several enzymes. These include

enzymes Biochemical function

 Cytochrome oxidase,
 catalase,
 tyrosinase,
 Superoxide dismutase,
 monoamine oxidase,
 ascorbic acid oxidase,
 ALA synthase,
 phenol oxidase,
 uricase
Copper is necessary for the synthesis of hemoglobin (Cu is a
constituent of ALA synthase, needed for heme synthesis).
Lysyl oxidase (a copper-containing enzyme) is required for the
conversion of certain lysine residues of collagen and elastin to
allysine which are necessary for cross-linking these structural
proteins.
 Ceruloplasmin serves as ferroxidase and is involved in the
conversion of iron from Fe2+ to Fe3+ in which form iron
(transferrin) is transported in plasma.
 5. Copper is necessary for the synthesis of melanin and
phospholipids.
 6. Development of bone and nervous system (myelin) requires Cu.
 7. Certain copper-containing non-enzymatic proteins have been
identified, although their functions are not clearly known. These
include
 hepatocuprein (storage form in liver)
 cerebrocuprein (in brain) and
 hemocuprein (in RBC).
 8. Hemocyanin, a copper protein complex in invertebrates,
functions like hemoglobin for O2 transport.
Dietary requirements
 Adults — 2-3 mg/day
 Infants and children — 0.5-2 mg/day

Sources
 Liver, kidney, meat, egg yolk, cereals, nuts and green leafy
vegetables.
 Milk is a poor source.
Absorption
 About 10% of dietary copper is absorbed, mainly in the
duodenum.
 Metallothionein is a transport protein that facilitates copper
absorption.
 Phytate, zinc and molybdenum decrease copper uptake
Plasma copper
 The copper concentration of plasma is about 100-200 mg/dl.
 Most of this (95%) is tightly bound to ceruloplasmin while a small
fraction (5%) is loosely held to albumin.
 Normal concentration of serum ceruloplasmin is 25-50 mg/dl. It
contains about 0.34% copper (6-8 atoms of Cu per molecule, half
in Cu2+ state and the other half in Cu+ state).
 Copper deficiency :

Disease states Severe deficiency of copper causes demineralization of bones,

demyelination of neural tissue, anemia, fragility of arteries,
myocardial fibrosis, hypopigmentation of skin, greying of hair.
Menke’s disease :
This disorder is due to a defect in the intestinal absorption of
copper.
It is possible that copper may be trapped by metallothionein in the
intestinal cells.
The symptoms of Menke’s disease include decreased copper in
plasma and urine, anemia and depigmentation of hair.
Wilson’s disease (hepatolenticular degeneration) :
It is a rare disorder (1 : 50,000)of abnormal copper metabolism and
is characterized by the following manifestations.
 ● Copper is deposited in abnormal amounts in liver and lenticular
nucleus of brain. This may lead to hepatic cirrhosis and brain
necrosis.
 ● Low levels of copper and ceruloplasmin in plasma (reference
range 20–50 mg/dl) with increased excretion of copper in urine.
 ● Copper deposition in kidney causes renal damage. This leads to
increased excretion of amino acids, glucose, peptides and
hemoglobin in urine.
 ● Intestinal absorption of copper is very high, about 4-6 times
higher than normal.
Probable causes of Wilson’s disease :
The following explanations are offered to understand the etiology
of this disease.
 1. A failure to synthesize ceruloplasmin or an impairment in the
binding capacity of copper tothis protein or both. As a result of
this, copper is free in the plasma which easily enters the tissues
(liver, brain, kidney), binds with the proteins and gets deposited.
The albumin bound copper is either normal or increased.
 2. A mutation in the gene encoding copper binding ATPase is
believed to be responsible for Wilson’s disease. Defect in this
ATPase reduces intestinal excretion of Cu through bile.
Treatment :
Administration of penicillamine, a naturally occurring copper
chelating agent, is used for the treatment of Wilson’s disease.
MANGANESE (Mn)
 The total body content of manganese is about 15 mg.
 The liver and kidney are rich in Mn.
 Within the cells, Mn is mainly found in the nuclei in association
with nucleic acids.
 1. Mn serves as a cofactor for several enzymes. These include
 arginase,
 pyruvate
Biochemical  carboxylase,
functions  isocitrate dehydrogenase,
 superoxide dismutase (mitochondrial) and
 peptidase.
 2. Mn is required for the formation of bone, proper reproduction
and normal functioning of nervous system.
 3. Mn is necessary for the synthesis of mucopolysaccharides and
glycoproteins.
 4. Hemoglobin synthesis involves Mn.
 5. Mn inhibits lipid peroxidation.
 6. Mn is necessary for cholesterol biosynthesis.
Sources
 Cereals, nuts, leafy vegetables and fruits.
 Tea is a rich source of Mn.

Absorption
 About 3-4% of dietary Mn is normally absorbed in the small
intestine.
 Iron inhibits Mn absorption.
Serum Mn
 Manganese in the serum is bound to a specific carrier protein—
transmagnanin (a 􀁠-globulin).
 The normal blood contains about 5-20 mg/dl.
Disease states
Mn deficiency in animals causes
 1. Retarded growth, bone deformities and, in severe deficiency,
sterility.
 2. Accumulation of fat in liver.
 3. Increased activity of serum alkaline phosphatase, and
 4. Diminished activity of 􀁠-cells of pancreas (low insulin).