The Electrophoretic α₁-Globulin Pattern of Serum in α₁-Antitrypsin Deficiency

Figures & data

Figure 1.  Paper electrophoretic protein pattern of sera from two patients with α1-antitrypsin deficiency and from a control. Upper: Case No. 2. Middle: Normal. Lower: Case No. 5.

Table 1.  Serum protein and lipid patterns in five patients with anti-alpha-1 antitrypsin deficiency with normal values for comparison. All quantitative values are expressed as g per 100 ml.

Case No.	Total prot.	Protein electrophoresis						Total Lipids	Electrophoretic α1-fraction	Haptoglobulin group
		Alb.	α1	α2	β1	β2	γ
1.	7.1	4.6	0.26	0.55	0.44	0.39	0.89	0.60	0.14	2–1
2.	6.7	3.9	0.25	0.70	0.35	0.29	1.23	0.66	0.19	2–1
3.	7.7	4.8	0.21	0.63	0.46	0.42	1.19	0.65	0.18	2–2
4.	7.4	4.5	0.30	0.65	0.51	0.43	1.01	0.53	0.23	2–1
5.	7.9	4.5	0.19	1.01	0.54	0.34	1.29	0.68	0.24	2–2
Normal values	6.8–	4.2–	0.23–	0.35–	0.40–	0.24–	0.75–	0.50–	0.15–
M ± 2 S.D.	7.8	5.3	0.38	0.60	0.65	0.42	1.30	0.95	0.28

Figure 2.  Agar-gel electrophoresis of serum from patient 4 (above) and a healthy subject (below). Both belong to haptoglobin group 2-2.

Figure 3.  Polysaccharide pattern after electrophoresis in a buffer of pH 4.5. The upper fraction is M1 i.e. mainly orosomucoid. Nr. 1. Abnormal. Normal. No. 5.

Figure 4.  Immunoelectrophoretic analysis of patient sera 1 to 5. Each serum was run with a normal serum (above the slit). The “specific” anti- α1-antitrypsin serum in the slit.

Table 2.  Immunological methods used to estimate the α1-Antitrypsin

Case No.	Immunoelectrophoresis according to			Ouchterlony's double diffusion Agar plates
	Scheidegger (Micro)		Grabar (Macro) Rabbit-a-Hs*	Absorbed Rabbit-a-Hs* Titer **
	Horse-a-Hs*	Absorbed Rabbit-a-Hs*
1	0	(+) trace	+ slow	1/6 (5)***
2	(+)-trace	+ *	? ****	1/8 (6)
3	0	(+) *	(+) slow	1/4 (3)
4	0	+ *	+ slow	1/8 (6)
5	0	(+) *	+ slow	1/3 (2)
Control	+++	+++	+++	1/128 (100)

* -a-Hs –antihuman serum; ** Dilution of serum giving comparable results; *** Figures within brackets represent calculated concentration in percent of normal; ****Technically unsatisfactory.