TABLE 10-3
M a jo r E ryth ro cyte M em b ra n e P ro te in s
*
Molecular Mass
(kDa)
Monomer
SDS
Gel
Band*
Protein
Gel
Calc
Molecules/
Cell,
Thousands
Oligomeric
State
Approximate
Proportion,
%
Peripheral
or
Integral
Chromosome
Location
Associated
Diseases**
1
a
Spectrin
240
281
242 ±20
Heterodimer/
14
P
Iq22-q25
HE,HPP,HS
tetramer/
oligomer
2
ß
Spectrin
2 2 0
246
242 ±20
13
P
14q23~q24.2
HE,HPP,HS
2 . 1
Ankyrin'
2 1 0
206
124 ±11
Monomer
6
'
P
8
pl
1 . 2
HS
2.9
a
Adducin
103
81
~30
Tetramer
< 1
P
4pl6.3
ß
Adducin
97
80
~30
< 1
P
—
—
3
AE1
90-100
1 0 2
- 1 2 0 0
Dimer or tetramer
29
I
17ql
2
-q
2 1
HS,SAO,HAc
4.1
Protein 4.1
80+78
6 6
- 2 0 0
Monomer
5
P
Ip33-p34.2
HE
4.2
Pallidin
72
77
-250
? Dimer or trimer
5
P
15q 15-q21
HS
4.9
Dematin'
48+52
43+46
-140
Trimer'
1
P
—
—
p55
55
53
-80
? Dimer
P
Xq28
—
5
ß
Actin
43
42
-500
Oligomer (-14)
6
P
7pter-q22
—
Tropomodulin
43
41
-30
Monomer
P
9q22
—
6
G3PD
35
36
-500
Tetramer
5
P
12pl3
—
7
Stomatin
31
32
—
—
4
I
9q34.1
?HSt
Tropomyosin
27+29
28
-70
Heterodimer'
P
lq31
—
8
Protein
8
23
—
- 2 0 0
—
1 - 2
P
—
—
PAS-1
Glycophorin A
36
14
- 1 0 0 0
Dimer
1 . 6
I
4q31
None
PAS-2
Glycophorin C
32
14
-
2 0 0
'
—
0 . 1
I
2ql4~q21
HE
PAS-3
Glycophorin B
2 0
8
- 2 0 0
?Dimer
0 . 2
I
4q31
None
Glycophorin D
23
1 1
- 2 0 0
—
0 . 0 2
I
2ql4~q21
HE
Glycophorin E
—
6
—
—
—
I
4q31
—
*Reproduced with permission from P. S. Becker and S. E. Lux: Hereditary Spherocytesis and Hereditary Elliptocytosis. In
T h e M e ta b o lic B a s is o f I n h e r ite d D is e a s e ,
7th Ed., C. R. Scriver,
A. L. Beaudet, W. S. Sly, et al„ Eds. McGraw-Hill, p. 3513, 1995.
**HE = Hereditary elliptocytosis; HPP = hereditary pyropykilocytosis; HS = hereditary spherocytosis; SAO = Southeast Asian ovalocytosis; HSt = hereditary stomatocytosis; HAc = hereditary
acanthocytosis.
