The normal response of the immune system to infections of the skin or mucosal tissues requires the ability of peripheral blood leukocytes to mobilize to the site of infection. In a classical inflammatory reaction, neutrophils and monocytes migrate in response to chemotactic factors released at the infection site. However, until recently, the molecular mechanisms which enable leukocytes to migrate across blood vessels have been largely unknown. These mechanisms have been elucidated, in part, by the characterization of a recently defined disease called human leukocyte adhesion deficiency (LAD). Patients with this heritable disease suffer from severe recurrent bacterial and fungal infections of soft tissues, primarily skin and mucous membranes. Infected, necrotic lesions in these patients contain few leukocytes despite the observation that these patients have chronic leukocytosis. In the early 1980's, several laboratories demonstrated that this disease is due to a cell surface deficiency of the LFA-1, Mac-1, and p150, 95 family of leukocyte adhesion proteins. More recently we have shown that LAD is due to heterogenous mutations in the β subunit common to the all three of these leukocyte glycoproteins. Although LAD is a rare disease, the analysis of this disease has greatly increased our understanding of the biology of the leukocyte adhesion proteins and the molecular mechanisms involved in the immune response to infections of the skin.

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SLE調節性T細胞サブセットおよび表面抗原についてモノクロナール抗体を用いて解析したところ, T3, T4, T8抗原陽性細胞数,抗原量,およびTPA処理後のmodulationは,正常と有意の差を示さなかった.しかし,サプレッサー・インデューサー細胞を固定する抗2H4抗体を用いて, T4+2H4+細胞を算定すると,活動期腎症で著明な低下が認められた.活動期にもかかわらず, T4+2H4+が正常であったものについて, AMLR刺激後の2H4抗原量を調べると,正常と異なり2H4抗原量が著明に低下していることが明らかとなつた.

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チトクロムb₅₅₈は大小2つのサブユニット(L, S)からなり,伴性劣性型CGDではLサブユニットが,常染色体劣性型CGDではSサブユニットがそれぞれ欠損している.単クローン抗体を用いた蛋白染色で,いずれの例でも両サブユニットがともにほとんど検出できなかった.
他に,生化学,免疫細胞化学のデータをもとにCGDを分類し,遺伝子治療の可能性についても述べた.

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