Atlas of Antinuclear Antibodies
■ Autoantibodies recognizing non-chromatin related proteins
DNA
precursor mRNA
(hnRNA)
RNA polymerase II
introns
introns
U2RNP
splicing
mRNA
U1RNP
U5RNP
U4/U6RNP
splicing
The genetic information of DNA is transcribed to precursor mRNA (heterogeneous nuclear RNA, hnRNA). The small nuclear
RNAs (small nuclear RNA, snRNA) such as U1, U2, U4/U6 and U5 RNA are involved in the splicing of hnRNA. These RNAs are
complexed with proteins to form ribonucleoproteins (RNPs).
Anti-U1RNP antibody
U1 RNA
Sm
B
B'
D
E
F
G
A
C
RNP
photo21
Pattern
Coarse speckled (larger granular) nuclear pattern with no nucleolar and cytoplasmic staining. The
chromosomal regions do not stain in mitotic cells.
14
Antigen
3 kinds of polypeptides bound to U1 RNA; U1RNP-68kD, U1RNP-A(34kD), U1RNP-C(23kD)
Other analytical
method
DID, ELISA, WB, Immunoprecipitation, CLEIA
Clinical
ignificance
The presence of the antibodies to U1RNP constitute one of major diagnostic criteria for MCTD.
References
15, 16
Autoantibodies recognizing non-chromatin related proteins
Copyright © 2016 MEDICAL & BIOLOGICAL LABORATORIES CO., LTD. All Rights Reserved.
Atlas of Antinuclear Antibodies
Anti-Sm antibody
Pattern
Coarse speckled staining similar to those of
anti-U1RNP antibody.
Antigen
U1, U2, U4/U6 and U5 RNA-bound proteins
with 5 kinds of epitopes, B'/B(29kD/28kD),
D(16kD), E(12kD), F(11kD) and G(10kD).
The main epitopes comprise B'/B and D
polypeptides. Antibodies to E-F-G complex
as dominant epitopes are also reported.
photo 22
AF/CDC-5*
Other
analytical
method
DID, ELISA, WB, Immunoprecipitation,
Clinical
significance
Marker antibody for SLE. The presence of
CLEIA
anti-Sm antibody is included in the criteria
for the classification of SLE by the American
College of Rheumatology.
References
17
Pattern
Coarse speckled nuclear staining similar to
Anti-U2RNP antibody / Anti-U1U2RNP antibody
those of anti-U1RNP antibody.
Antigen
U2RNA-bound proteins with 2 kinds of
epitopes on A'(32kD) and B"(28.5kD).
Other
analytical
method
Immunoprecipitation
Clinical
significance
Polymyositis/scleroderma overlap syndrome
References
3, 15, 16
photo 23
* Reference serum from Centers for Disease Control
SLE: systemic lupus erythematosus, SSc: systemic sclerosis, MCTD: mixed connective tissue disease, SS: Sjögren's syndrome, PM/DM:
polymyositis/dermatomyositis, RA: rheumatoid arthritis, PBC: primary biliary cirrhosis, AIH: autoimmune hepatitis
Autoantibodies recognizing non-chromatin related proteins 15
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Atlas of Antinuclear Antibodies
Anti-hnRNP antibody
Pattern
Large speckled (granular) nucleoplasmic
staining with no chromosomal fluorescence in
mitotic cells. The antibodies frequently coexist
with anti-U1RNP and anti-Sm antibodies, and
give coarse speckled patterns.
Antigen
hnRNA bound proteins-A/B, A1, A2, C, I
Other analytical
method
ELISA, WB
Clinical
significance
These antibodies were first reported in
photo 24
patients with MCTD. Antibodies to the A/
B polypeptides and those to the A1 and A2
polypeptides occur in SLE and rheumatoid
arthritis, respectively. Recently, antihnRNP-I antibodies with weak nuclear
staining specific for scleroderma and antihnRNP-C antibodies with speckled nuclear
staining in scleroderma were reported.
References
18, 19, 20, 21
Anti-SS-A/Ro antibody
Y-RNA
Ro 60 kD
Ro 52 kD
SS-B/La
La
SS-A/Ro
photo 2　F/CDC-7*
Pattern
Fine speckled nuclear staining observed in acetone-fixed slides, but not in alcohol-fixed ones.
The chromosomal region shows no fluorescence in mitotic cells.
Antigen
Y1-Y5RNA-bound proteins (52kD, 60kD)
Other analytical method
DID, ELISA, WB, Immunoprecipitation, CLEIA
Clinical significance
These antibodies occur most frequently in patients with SS, and those with SLE. Sometimes
found in patients with rheumatoid arthritis, myositis or scleroderma. The presence of the
antibodies in the mother is associated with neonatal lupus and congenital heart block. The
presence of anti-SS-A antibodies constitutes one of the diagnostic criterion of SS in Europe.
References
16
22
Autoantibodies recognizing non-chromatin related proteins
Copyright © 2016 MEDICAL & BIOLOGICAL LABORATORIES CO., LTD. All Rights Reserved.
Atlas of Antinuclear Antibodies
Anti-SS-B/La antibody
Pattern
Fine dense speckled nuclear staining
is observed in interphase cells with no
chromosomal fluorescence in mitotic cells.
Antigen
48 kD SS-B/La protein, a termination factor
for RNA polymerase III transcription
Other
analytical
method
DID, ELISA, WB, Immunoprecipitation,
Clinical
significance
The antibodies are found in patients with SS
CLEIA
and SLE. As with anti-SS-A antibodies, the
presence of these antibodies is associated
photo 26
with neonatal lupus and congenital heart
AF/CDC-2*
block. The antibodies were reported to be
frequently positive in patients with recurrent
annular erythema. The presence of antiSS-B antibodies constitutes one of the
diagnostic criterion of SS in Europe.
References
23
Pattern
Granular or few nuclear dots staining**, 0 to 6
Anti-p80 coilin antibody
dots (ave. 2 dots) per nucleus fluorescence.
The distinct large dotty fluorescence can be
seen in cells during S and G2 phases, and
mitotic cells show no staining.
Antigen
p80 coilin, the 80kD protein composing the
coiled body in nucleoplasm.
photo 27
Other
analytical
method
WB
Clinical
significance
These antibodies occur in some patients
with SS and those with primary biliary
cirrhosis.
References
24, 25
* Reference serum from Centers for Disease Control
SLE: systemic lupus erythematosus, SSc: systemic sclerosis, MCTD: mixed connective tissue disease, SS: Sjögren's syndrome, PM/DM:
polymyositis/dermatomyositis, RA: rheumatoid arthritis, PBC: primary biliary cirrhosis, AIH: autoimmune hepatitis
Autoantibodies recognizing non-chromatin related proteins 17
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Atlas of Antinuclear Antibodies
Anti-Sp100 antibodies
Pattern
Granular or multiple (1 to 24) nuclear dots
staining**.
Antigen
Sp100 protein. Multiple nuclear dots
staining is also observed by the antibodies
to promyelocytic leukemia (PML) protein,
NDP53 (a binding protein to PML oncogenic
domain including Sp100).
photo 28
Other analytical
method
WB
Clinical
significance
These antibodies occur in some patients
References
26, 27, 28
with PBC.
* Reference serum from Centers for Disease Control
** Since the numbers of staining dots seen in few nuclear dots and multiple nuclear dots patterns overlap each other, it is sometimes difficult to
distinguish these patterns. These patterns are often called granular or nuclear dots patterns.
18
Autoantibodies recognizing non-chromatin related proteins
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