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Astrocytic deformity and globular structures are characteristic of the brains of patients with aceruloplasminemia
Kaneko K, Yoshida K, Arima K, Ohara S, Miyajima H, Kato T, Ohta M, Ikeda SI.. Journal of neuropathology and experimental neurology, 2002-12-01
Topic: Brain proteins, microglia and lab models
Aceruloplasminemia is an interesting disease, the study of which helps elucidate how iron-induced oxidative stress is involved in neuronal cell death. In order to study the neuropathological characteristics associated with oxidative stress, we scrutinized the brains of 5 patients with aceruloplasminemia histopathologically and immunohistochemically. The pathological findings were essentially similar in all patients. In the frontal cortices, iron deposition and neuronal cell loss were trivial, but in the basal ganglia (especially in the caudate nucleus and putamen), severe iron overload and extensive neuronal loss were noted. Iron deposition was more prominent in the astrocytes than in the neurons in both regions. 4-hydroxynonenal (HNE), one of the most physiologically active lipid peroxides, was strongly detected on neurons and astrocytes by immunostaining. Markedly deformed astrocytes were observed in the striatum. These astrocytes were similar to Alzheimer type 1 astrocytes. Globular structures were seen in proportion to the degree of iron deposition. They clearly reacted with anti-glial fibrillary acidic protein (GFAP) and anti-S-100 antibodies and contained glial fibril-like filaments, but showed no or only faint immunoreactivity to antibodies for neuronal marker proteins, such as neurofilament and synaptophysin. Therefore, the globular structures presumably originated from astrocytes. The structures also reacted positively to anti-HNE and anti-ubiquitin antibodies. We conclude that astrocytic deformities and globular structures are characteristic neuropathological features of aceruloplasminemia and are closely linked to iron overload and subsequent oxidative stress.
Key findings
- In order to study the neuropathological characteristics associated with oxidative stress, we scrutinized the brains of 5 patients with aceruloplasminemia histopathologically and immunohistochemically.
- We conclude that astrocytic deformities and globular structures are characteristic neuropathological features of aceruloplasminemia and are closely linked to iron overload and subsequent oxidative stress.
Why it matters
In five patients with aceruloplasminemia, a disease of iron overload, deformed astrocytes and globular structures were found in the brain and linked to iron deposition and oxidative stress. This is a study of a rare iron metabolism disorder, not Alzheimer's disease, so it does not directly inform Alzheimer's treatment or prevention.
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Study details
- Evidence strengthLow
How this study scored 34/100
Published in a peer-reviewed journal. Scored zero for study design, sample size, effect reported, studied in people and randomised / blinded because the record does not state it. We have no data on senior author's track record, so that was left out: it earned 33 of the 96 points we could judge it on, which is 34 out of 100.
| Component | Points | Why |
|---|---|---|
| Journal tier | 7 / 10 | Tier 3 — peer-reviewed journal (Journal of neuropathology and experimental neurology) |
| Journal's citation record | 2 / 4 | Journal of Neuropathology & Experimental Neurology: journal h-index 239 (OpenAlex, 2026-10-29) |
| Study design | 0 / 12 | No study design recorded — scores zero, not guessed (not stated in the record — scored 0) |
| Strength of evidence | 1 / 5 | Rated “low” in our summary |
| Peer-review validation | 10 / 10 | Peer-reviewed. Published as an article in a named peer-reviewed journal. |
| How recent | 1 / 5 | 2002 — older than 20 years, still counted |
| Sample size | 0 / 4 | Participant count not stated — scores zero (not stated in the record — scored 0) |
| Effect reported | 0 / 3 | No effect size reported — scores zero (not stated in the record — scored 0) |
| Studied in people | 0 / 3 | Not stated whether this was studied in people — scores zero (not stated in the record — scored 0) |
| Randomised / blinded | 0 / 2 | No randomisation or blinding stated — scores zero (not stated in the record — scored 0) |
| Times cited | 4 / 7 | Cited 62 times (OpenAlex, 2026-10-10) |
| Citations per year | 1 / 5 | 2.4 citations a year over 26 year(s) since 2002 |
| Impact for its field and year | 2 / 6 | Cited 1.5× the average for papers of its field and year (OpenAlex FWCI) |
| Cited by studies here | 1 / 6 | Cited by 1 other studies in this library |
| Still being cited | 0 / 4 | Cited 1 times in 2024–2026 (2026 still in progress; OpenAlex) |
| Senior author's track record | — / 4 | OpenAlex's profile for last-listed author Shu-ich Ikeda holds only 6 work(s), too few to judge a track record — left out of this score |
| Institution's track record | 0 / 3 | Shinshu University: institution h-index 440 (OpenAlex) |
| Multi-centre collaboration | 2 / 3 | OpenAlex lists 7 institution(s) in 1 country |
| Indexed in PubMed | 2 / 2 | Indexed in PubMed (PMID 12484569) |
| Free to read | 0 / 2 | Behind a paywall — scores zero |
| Points | 33 / 96 | Scored on 19 of 20 parts. We have no data for the rest, so they are left out: 33 of the 96 points we could judge it on is 34 out of 100. |
| Total | 34 / 100 | — |
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Cite this study APA, Vancouver, BibTeX · download .bib or .ris
Built from the record alzhub holds for this paper, not looked up. Volume, issue and page numbers are not held in the record. The record names 8 authors; APA and Vancouver show the first six, BibTeX and the downloads carry all of them. Check the citation against the original before you use it.
APA 7th
Kaneko, K., Yoshida, K., Arima, K., Ohara, S., Miyajima, H., Kato, T., et al. (2002). Astrocytic deformity and globular structures are characteristic of the brains of patients with aceruloplasminemia. Journal of neuropathology and experimental neurology. https://doi.org/10.1093/jnen/61.12.1069
Vancouver
Kaneko K, Yoshida K, Arima K, Ohara S, Miyajima H, Kato T, et al. Astrocytic deformity and globular structures are characteristic of the brains of patients with aceruloplasminemia. Journal of neuropathology and experimental neurology. 2002. doi:10.1093/jnen/61.12.1069. PMID: 12484569.
BibTeX
@article{kaneko2002-12484569,
author = {Kaneko, K. and Yoshida, K. and Arima, K. and Ohara, S. and Miyajima, H. and Kato, T. and Ohta, M. and Ikeda, S. I.},
title = {{Astrocytic deformity and globular structures are characteristic of the brains of patients with aceruloplasminemia}},
journal = {Journal of neuropathology and experimental neurology},
year = {2002},
doi = {10.1093/jnen/61.12.1069},
pmid = {12484569},
url = {https://doi.org/10.1093/jnen/61.12.1069}
}
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