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Fluorescence patterns of nuclear autoantigens

Autoantibodies recognizing nucleolus related proteins




nucleolus image


The nucleolus is composed of the central fibrillar region and the outer granular component. In the fibrillar center, nucleolus organizer region (NOR) is related to the reformation of the nucleolus after cell divison.


Anti-U3RNP antibody (Anti-fibrillarin antibody)


photo 29


Pattern Clumpy nucleolar staining in interphase cells with chromosomal region fluorescence of mitotic cells.
Antigen U3RNA-bound protein; fibrillarin (34kD)
Other analytical method Immunoprecipitation
Clinical significance These antibodies occur in SSc (mainly observed in patients with diffuse cutaneous scleroderma not associated with arthritis and lung fibrosis), and in hepatocellular carcinoma.
References 29, 30

Anti-RNA polymerase I antibody


photo 30


Pattern Punctate or speckled nucleolar staining in interphase cells. The chromosomal regions show no staining in mitotic cells.
Antigen RNA polymerase I, II and III component proteins (more than 10 proteins ranged from 12.5 to 210kD). The autoantibodies targeting RNA polymerase II or III, and those reacting to more than one RNA polymerase were reported.
Other analytical method Immunoprecipitation
Clinical significance These antibodies occur in patients with SSc, frequently associated with patients with severe internal organ involvement (mainly lung and kidney) and poor prognosis.
References 4, 12, 29



Anti-7-2RNP antibody (Anti-Th antibody)



photo 31


Pattern Dotty nucleolar staining
Antigen RNaseP(8-2RNP), RNaseMRP(7-2RNP)
Other analytical method Immunoprecipitation
Clinical significance These antibodies occur in patients with scleroderma, mainly associated with limited cutaneous involvement.
References 29



Anti-PM-Scl antibody


photo 32


Pattern Homogeneous or diffuse nucleolar staining with no chromosomal staining in mitotic cells.
Antigen The protein complex of more than 10 nucleolar proteins including 75kD and 100kD polypeptides.
Clinical significance These antibodies occur in patients with polymyositis/scleroderma overlap syndrome in Europe and America, but not in Japan.
References 29



Anti-NOR-90 antibody


photo 33


Pattern Coarse speckled or granular nucleolar staining in interphase cells. Several intensely stained dots can be seen in the chromosomal regions in mitotic cells.
Antigen 90kD/92kD proteins in nucleolus organizer region, recognizing RNA polymerase I transcription factor, hUBF (human upstream binding factor) in the fibrillar center.
Other analytical method DID, ELISA, WB, Immunoprecipitation
Clinical significance These antibodies occur in patients with SSc frequently associated with Raynaud's phenomenon, also those with hepatocellular carcinoma and SS in Japan.
References 30, 31



Anti-RNA helicase antibody


photo 34


Pattern Dense speckled nucleolar staining with no chromosomal staining in mitotic cells.
Antigen 100 kD nucleolar RNA helicase protein
Other analytical method Immunoprecipitation
Clinical significance These antibodies occur in patients with SSc, SLE or watermelon stomach disease.
References 32, 33



Other autoantibodies to nucleolar antigens:
Anti-B23/nucleophosmin antibodies occur in patients with hepatocellular carcinoma and show homogeneous nucleolar staining. [References: 30, 34]
Anti-No55 antibodies occur in patients with interstitial cystitis and show homogeneous nucleolar pattern. [Reference: 35]



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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


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