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Chorea-acanthocytosis Abnormal composition of covalently bound fatty acids of erythrocyte membrane proteins.

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Chorea-Acanthocytosis: Abnormal
Composition of Covalently Bound Fatty
Acids of Erythrocyte Membrane Proteins
Tetsuo Sakai, MD," Yasunobu Antoku, MD," Hiroshi Iwashita, MD," Ikuo Goto, MD,t
Keiji Nagamatsu, MD,$ and Hiroalu Shii, MDO
Phospholipid class, peak profile of each phospholipid class, loosely bound fatty acids, covalently (tightly) bound
fatty acids of the erythrocyte membranes, and plasma fatty acids were investigated using high-performance liquid
chromatography in six patients with chorea-acanthocytosis and 14 age- and sex-matched normal control subjects.
Additionally, six patients with Huntington's disease were included as disease control subjects in the study of covalently
bound fatty acids. Study of covalently (tightly) bound fatty acids in erythrocyte membrane proteins after alkaline
hydrolysis, hitherto undescribed in chorea-acanthocytosis, revealed that palmitic acid (C 16:0) was significantly increased and stearic acid (C18: 0) was decreased in the patients with chorea-acanthocytosis. Analyses for total covalently
bound fatty acids disclosed that palmitic and docosahexaenoic (C22:6) acids were increased and stearic acid was
decreased in chorea-acanthocytosis. Phospholipid class (phosphatidylcholine, phosphatidylethanolamine,sphingomyelin, and phosphatidylserine) and peak profile of each phospholipid class from the erythrocyte membranes did not differ
between the patients with chorea-acanthocytosis and the control subjects. Of the loosely bound fatty acids, linoleic
acid (C18: 2) was significantly decreased in those with chorea-acanthocytosis, which seemed to be nonspecific.
Sakai T, Antoku Y , Iwashita H, Goto I, Nagamatsu K, Shii H. Chorea-acanthocytosis.
abnormal composition of covalently bound fatty acids of erythrocyte
membrane proteins. Ann Neurol 1991;29:664-669
Chorea-acanthocytosis or Levine-Critchley syndrome
was first described in a New England family [I, 2).
The disease was characterized by (1) adult onset of
symptoms, (2) progressive orofacial dyskinesia and
choreic movements of the limbs, (3) tongue o r lip biting or both, (4) peripheral neuropathy, ( 5 ) acanthocytosis in peripheral blood and normal plasma p-lipoprotein, (6) an increased level of serum creatine kinase,
and (7) autosomal recessive inheritance, if it is a genetic
disease {3].
The pathogenesis of chorea-acanthocytosis remains
to be elucidated. The findings of deformed erythrocytes (acanthocytes) and of increased serum creatine
kinase (mainly composed of MM isozyme) led to the
hypothesis of a generalized membrane defect {4].The
finding that incubation of normal control erythrocytes
with patient serum fails to induce acanthocytosis [ S - 7 )
suggests that the abnormality exists in the erythrocyte
membranes. Many investigations of lipids, structural
proteins, and enzymes of erythrocytes in choreaacanthocytosis have been unrewarding.
In the present study, covalently (tightly) bound fatty
acids or acylproteins, hitherto undescribed, as well as
phospholipid class and loosely bound fatty acids of
erythrocyte membranes, were investigated in patients
with chorea-acanthocytosis, patients with Huntington's
disease as disease control subjects, and normal control
From the 'Department of Neurology, National Chikugo Hospital,
Fukuoka; tDepartment of Neurolom. Neurological Institute, Facd t y of Medicine, Kyushu Univers&, Fukuoka; $Department of
Neurology, Oita Prefectural Hospital, Oita; and &Departmentof
Neurology, Kokura Memorial Hospital, Fukuoka, Japan.
Received Aug 2, 1990, and in revised form Sep 4, Nov 5 , and Dec
4.Accepted for Dubkation Dec 17. 1990.
Materials and Methods
Six patients with chorea-acanthocytosis (male-female ratio,
4:2; aged 36 to 49 years; mean age, 43 years) and 6 patients
with Huntington's disease (male-female ratio, 4:2; aged 29
to 59 years; mean age, 46 years) were selected according to
the diagnostic criteria proposed previously [3}. At the time
blood was drawn, 1 of 6 patients with chorea-acanthocytosis,
and 4 of 6 patients with Huntington's disease were taking
medication such as sulpiride and trihexyphenidyl. Fourteen
healthy volunteers (male-female ratio, 8:6; aged 29 to 63
years; mean age, 46 years) were chosen in order to match
two volunteers by age and sex with each patient in the study.
Fasting blood was drawn from all the subjects in the morning,
heparinized, and immediately cooled in ice water. Erythro-
To Dr sakai, Department of Neurology,
National Chikugo Hospital, 515 Kurakazu, Chikugo City, 833 Fukuoka pref., Japan.
664 Copyright 0 1991 by the American Neurological Association
Table I . Phospholipid Class of Erythrocyte iMembranes"
Phosp hatidylcholine
Phosphatid ylserine
C-A (n = 5 )
(n = 10)
17.4 t 3.09
46.9 t 4.18
28.7 f 6.95
17.9 t 3.78
43.8 f 5.98
"Values (area percentage) are means
C-A = chorea-acanthocytosis.
cyte membranes were prepared as described previously 181
and stored at - 80°C until use.
Analyses of Phospholipids
hpids were extracted from the membrane suspension according to Folch and colleagues [9]and the lower chloroform
phase was evaporated to dryness in a stream of nitrogen gas
and dissolved in ethanol. An aliquot of solution was applied
to high-performance liquid chromatography (HPLC). HPLC
analyses of phospholipids were performed with a Shimadzu
L C 4 A system (Shimadzu, Kyoto, Japan) equipped with an
automatic sample injector and a spectrophotometric detector.
Analytical conditions of HPLC were the same as those described in a previous report [lo].
AnaJyses of Fatty Acids
Lipids were extracted from 1 ml of membrane suspension by
the method of Folch and colleagues [9] and, thereafter,
loosely bound fatty acids were obtained according to Marinetti and associates (11).The delipidized protein pellets were
suspended in potassium hydroxide (KOH) in methanol, saponified by heating at 98°C for 20 minutes, and acidified
with hydrochloric acid (HCl), and then fatty acids were extracted with chloroform and dried in a stream of nitrogen
gas. The fatty acids thus obtained (covalently bound fatty
acids after alkaline hydrolysis) were esterified with a fluorescent labeling agent, 9-anthryldiazomethane (Funakoshi Drug,
Tokyo, Japan). Total covalently bound fatty acids were obtained as follows: the delipidized protein pellets, acquired by
the method of Marinetti and associates [ 1I), were hydrolyzed
with 0.5 M HCl in a mixture of acetonitrile and water for
45 minutes at 98"C, and free fatty acids were dissolved in
chloroform, dried [12, 131, and esterified with 9-anthryldiazomethane. Analytical HPLC conditions of the fatty acids
were as described previously 114, 151. All chemicals were of
HPLC grade; butylated hydroxytoluene was included as an
antioxidant in the chloroform and methanol at a concentration of 5 mg/100 ml.
Statistical analyses were carried out with Student's t tests.
Phospholipid Classes of Erythrocyte Membranes
There was no significant difference in area percentage
of four phospholipids (phosphatidylcholine [PC},
phosphatidylethanolaine {PE}, sphingornyelin [SM],
and phosphatidylserine [PSI) between the patients with
mi n
Fig I . Separation of evythroryte membrane phospholipids by
high-performance liquid chromatography. Phosphatidylcholine
(PC), phoJphatidylethanolamine (PE), and sphingomyelin (SM)
were separated into three, six, and two peaks, respectively, on the
chromatogram. Three peaks of PC, the anterior two peaks
inumbered 1 on the chromatogram) and posterior four peak- (2
on the chromatogrum) of PE, and two peak of SM were compared between patients with chorea-acanthocytosisand control
Table 2. Peak Projle of Each Phospholipid
fmm Evythrocyte iMembranes"
C-A (n = 5 )
Control Subjects (n = 10)
80.8 k 6.87
8.8 ? 2.9
10.8 4.09
80.2 +- 9.40
8.3 +- 3.9
11.5 -+- 5.70
43.7 _c 4.79
56.3 2 4.78
2 6.7
31 -+- 6.6
* 5.6
The numbers for PC and PE correspond to those in Figure 1
"Values (area percentage) are means t SD.
C-A = chorea-acanthocytosis; PC = phosphatidylcholine; PE
phosphatidylethanolamine; SM = sphingomyelin.
chorea-acanthocytosis and the normal control subjects
(Table 1). Our newly devised method of HPLC El01
revealed that PC was divided into at least three peaks;
PE, into two peaks; and SM, into two peaks (Fig 1).
Therefore, area percentage of each peak from PC, PE,
and SM was compared between the patients with
chorea-acanthocytosis and the normal control subjects,
but there was no significant difference (Table 2).
Looseb Bound Fatty Acids of Elythrocyte Membranes
Fourteen fatty acids-C18 :3 , C14 : 0, C22 :6, C20: 4,
C18:2, C16:0, C18: 1, C18:0, C20:0, C22:1, C22:0,
Sakai et al: Chorea-Acanthocytosis 665
Table 3. Composition of Loosely Boznd Fatty Acids from Erythrocyte Membranes"
C-A (n
C22 :6
C18 :OK18 :1
C24:l/C24 : O
Saturation index
Control Subjects (n
HD (n = 6)
10.8 2 3.18
12.6 f 2.81
8.66 1.14d
25.7 t 1.42
12.5 2 1.54
16.0 f 0.47
10.4 t 1.29
12.2 t 0.93
11.2 2 1.02
25.1 + 0.62
13.1 f 1.17
15.8 t 0.54
8.17 -+ 2.151b
13.8 t 0.79'
12.1 t 2.01
25.4 t 1.34
12.7 f 0.89
15.8 2 0.34
1.30 t 0.15
1.10 2 0.14
0.65 0.05'
1.21 t 0.14
1.11 t 0.12
0.71 ? 0.03
1.25 t 0.11
1.28 L 0.22
0.72 2 0.04
"Values (area percentage) 'art! means
" p < 0.02.
' p < 0.005.
np < 0.001.
C-A = chorea-acanthocytosis; HD = Huntington's disease.
Table 4. Cornpositton of Covalently Boirnd Fiatty Acids after
Alkaline Hydrolysis from Erythrocyte Membranesa
C-A (n
3.99 +- 1.21
C22 :6
C18 :0
Control Subjects
(n = 10)
23.6 t 1.52
8.20 t 1.09
21.3 f 0.73b
10.8 & 1.21
31.4 5 LA&
3.14 2 0.77
24.1 t 1.48
9.18 2 1.18
18.8 2 1.46
10.0 2 0.75
34 1 f 1.86
0.68 i O.Obd
C16 : 0iC18 : 0
* 0.07
"Values (area percentage) are means 2 SD.
"p < 0.03.
cp < 0.02.
C24 :1, C24 :0, and C26 :0-were
identified in
sequence of elution. Linoleic acid (C18:2) was significantly decreased in the patients with choreaacanthocytosis ( p < 0.001), but other fatty acids were
not significantly different between the patients with
chorea-acanthocytosis and the normal control subjects. Docosahexaenoic acid (C22 : 6) was significantly
decreased and arachidonic acid (C20 : 4 ) was increased
in the patients with Huntington's disease, compared
with normal control subjects (Table 3; six major fatty
acids are described, and others are omitted).
CwaJently Bound Fatty Acids after
Alkaline Hydrolplsi'
Covalently bound fatty acids after alkaline hydrolysis
were composed of 10 fatty acids. Palmitic acid (C 16 :0)
was significantly increased (p < 0.05), and stearic acid
(C 18 : 0) was decreased (p < 0.02) in the patients with
chorea-acanthocytosis (Table 4; six major fatty acids
666 Annals of Neurology Vol 29 No 6 June 1991
- LI
dp < 0.005.
V Controls
Fig 2. C16:0iC18:0 ratios of covalently bound fatty acids
after crlkaline hydr&sis of erythrocyte membrane proteins from
patients with chorea-acantbocytosis (C-A) and control mbjects.
?'he CJ6:01C18:0 ratioJ were significantly increased in the
patients (0.68 f 0.061, compared with those in normal controls
10.55 0.071.
are listed in the table and others are omitted). Ratios
of Cl6 : 0 to C18 :0 were markedly increased in the
patienrs with chorea-acanthocytosis (p < 0.005) and
plotted ratios of chorea-acanthocytosis only overlapped
with 1 normal control subject (Fig 2).
Total CavaJently Bomd Fatty Acids
Eight fatty acids-C22:6,
C20:4, C18:2, C16:0,
C 18 :1, C 18:0, C24 : 1, and C24 :0-were identified in
order of elution. Docosahexaenoic acid (p < 0.005)
and palmitic acid (p < 0.01) were significantly in-
Table 5. Composition of Total Covalently Bound
Fatty Acids from E yythrocyte Membranes”
Table 6. Compo.rition of Plasma Fatty Avidsa
Control Subjects
Control Subjects
C-A (n = 6)
(n = 12)
H D ( n = 6)
4.09 f 1.02b 2.58 .t 0.55
C20:4 23.10 f 3.60 24.40 f 1.46
9.41 k 0.79
8.33 t 1.64
C16:O 19.60 ? 1.51d 17.20 2 1.40
8.86 t 0.48
8.77 t 0.96
C18:O 32.80 f 2.58‘ 35.30 k 1.43
1.88 f 0.53‘
24.0 f 1.89
10.60 t 2.75
16.60 t 1.65
9.55 ? 0.61‘
35.0 f 2.14
“values (area percentage) are means f SD.
bp < 0.005.
‘ p < 0.05.
dp < 0.01.
‘p < 0.02
C-A = chorea-acanthocytosis;HD = Huntington’s disease.
creased, and stearic acid (p < 0.05) was decreased in
the patients with chorea-acanthocytosis, as compared
to normal control subjects, whereas docosahexaenoic
acid (p < 0.05) was significantly decreased and oleic
acid (C18:l; p < 0.02) was increased in the patients
with Huntington’s disease, compared with normal control subjects (Table 5 ; six major fatty acids are listed).
Plasma Fatty Acids
Linoleic acid (p < 0.05) was significantly decreased and
oleic acid (p < 0.05) was increased in patients with
chorea-acanthocytosis, in comparison with normal volunteers, while there was no difference of plasma fatty
acid composition between the patients with Huntington’s disease and normal control subjects (Table 6; six
major fatty acids are in the table, but others are
The present study shows no significant differences of
area percentage of PC, PE, SM, and PS and of each
peak in PC, PE, and SM between the patients with
chorea-acanthocytosis and control subjects. This is consistent with findings of previous studies [l, 5 , 16-21).
Also, the present investigation, using HPLC instead
of gas chromatography, shows that only linoleic acid
(C18 :2 ) among the 14 loosely bound fatty acids examined was significantly decreased in the patients with
chorea-acanthocytosis. This supports the result reported by Bird and coworkers [ S ] . Since the decrease
in linoleic acid has been described in chronic debilitating diseases such as myotonic dystrophy [14} and
multiple scierosis [227, it seems to be nonspecific.
However, the present study shows an abnormal composition of covalently bound fatty acids in erythrocyte
membrane proteins from patients with choreaacanthocytosis. These findings are new. First, the covalently bound fatty acids after alkaline hydrolysis, which
C-A (n = 6)
(n = 14)
HD (n = 6)
7.37 & 1.43
9.83 f 0.89
22.9 f 4.20’
28.1 f 2.80
21.8 .t 2.34’
7.90 .t 0.83
7.57 ? 1.86
9.25 2 0.67
27.0 =k 2.54
26.6 f 1.63
18.5 f 2.87
8.43 f 0.70
5.78 f 2.20
9.23 t 1.46
28.4 f 3.62
25.1 .t 1.96
18.0 t 2.25
8.11 f 0.95
“Values (area percentage) are means ? SD.
bp < 0.05.
C-A = chorea-acanthocytosis;HD
Huntington’s disease.
were reported to be labile to mild alkaline hydrolysis
and to comprise 50 to 60% of total covalently bound
fatty acids according to Marinetti and associates ill], in
the patients with chorea-acanthocytosis differed from
those in normal control subjects. Second, palmitic and
docosahexaenoic acids were, in the analyses for total
covalently bound fatty acids, significantly increased and
stearic acid was decreased in the patients with choreaacanthocytosis compared to normal volunteers.
Covalently (tightly) bound fatty acids or acylproteins
were, in the last decade, postulated to be one of the
posttranslational modifications to the membrane proteins, and have been demonstrated in many kinds of
biological membranes or cytoskeleton proteins such as
erythrocyte membrane proteins [ll, 23, 241, acetylcholine receptor in cultured muscle cells 1251, vinculin
in chicken embryo fibroblasts 1261, and myelin proteins [27-29). Covalently bound fatty acids, which cannot be removed from the membrane proteins either
by strong detergents or by chloroform/methanol extraction and which are generally thought to be linked
to membrane proteins by carboxylic ester linkages (labile to mild alkaline hydrolysis) or by amide bond (stable to mild alkaline hydrolysis) Ell, 301, may influence
the shape, elasticity, and stability of the erythrocytes
via the modulation of the interaction of membrane proteins such as ankyrin with the plasma membrane [24).
To interpret abnormalities of covalently bound fatty
acids, we must pay attention to the report that at least
some of the covalently bound fatty acids of the erythrocyte membranes are dynamically acylated or deacylated
13 11, influenced by the surrounding environment, for
example, loosely bound fatty acids and plasma fatty
acids. The abnormal composition of total covalently
bound fatty acids in chorea-acanthocytosis was not inA uenced by abnormality of loosely bound fatty acids or
by abnormalities of plasma fatty acids. Morever, drug
effect, if any, should be negligible, since 5 of 6 patients
with chorea-acanthocytosis were not taking medication
at the time blood was drawn. Abnormalities of covalently bound fatty acids in chorea-acanthocytosis have
Sakai et al: Chorea-Acanthocytosis 667
not been described in other diseases such as myotonic
dystrophy [14), adrenoleukodystrophy (Y. Antoku et
al; unpublished data, 1985), or Huntington's disease
(present study), suggesting that these abnormalities
may be specific to chorea-acanthocytosis. Further studies will be necessary to clarify the question of whether
the membrane defect, demonstrated in the present
study, might be reflected in the central and peripheral
nervous systems and muscle.
Another classic disease associated with acanthocytosis is Bassen-Kornzweig syndrome, in which many
investigations of lipids have been made. Increased ratio
of PC to SM and profound deficiency of linoleic acid
from erythrocyte membranes have been reported in
Bassen-Kornzweig syndrome [32). The former abnormality of phospholipid distribution was not found in
patients with chorea-acanthocytosis, but the latter was
recognized in chorea-acanthocytosis and was thought
to be nonspecific. Study of covalently bound fatty acids
has not been reported so far in Bassen-Kornzweig syndrome.
The abnormal composition of covalently bound fatty
acids in chorea-acanthocytosis suggests abnormahties
in the functional roles that covalently bound fatty acids
should play in erythrocyte membrane. One of the resulting events could be disorganization of membrane
protein networks, leading to deformed erythrocytes
such as acanthocytes. Another might be release of
some structural proteins from erythrocyte membranes
[18, 191, since one of the major roles of covalently
bound fatty acids in the biological membranes is postulated to be anchorage of membrane proteins to the
lipid bilayer of biological membranes [ll, 30).
This study was supported by grants from the CNS Degenerative
Disease Research Committee, the Ministry of Health and Welfare,
This study is dedicated to Yoshigoro Kuroiwa, MD, now deceased,
formerly Professor of Department of Neurology, Neurological Institute, Kyushu University, who gave us the opportunity to see the
patients with chorea-acanthocytosisand encouraged us to do research
on the pathogenesis of the disease.
W e would like to express our gratitude to the subjects and family
members of both chorea-acanthocytosis and Huntington disease;
Shouzou Tobimatsu, MD, Department of Neurophysiology, Neurological Institute, Faculty of Medicine, Kyushu University, Fukuoka;
and Kouhei Miyazawa, MD, Kumamoto Psychiatric Hospital, Kumamoto, Japan for their kind cooperation.
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