Hemoglobin Brockton (. beta. 138 (H16) Ala yields Pro): An unstable variatn near the C-terminus of the. beta. -subunits with normal oxygen-binding properties

Journal Article · Tue Oct 04 04:00:00 EDT 1988 · Biochemistry; (USA)

DOI:https://doi.org/10.1021/bi00420a007· OSTI ID:6941073

Moo-Penn, W F; Jue, D L; Johnson, M H ^[1]; Olsen, K W ^[2]; Shih, D; Jones, R T ^[3]; Lux, S E ^[4]; Rodgers, P

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1. Centers for Disease Control, Atlanta, GA (USA)
2. Loyola Univ. of Chicago, IL (USA)
3. Oregon Health Sciences Univ., Portland (USA)
4. Children's Hospital Medical Center, Boston, MA (USA)

Hemoglobin Brockton ({beta}138 (H16) Ala {yields} Pro) is an unstable variant associated with a mild anemia. It has the same electrophoretic mobility as and cannot be resolved from Hb A. Oxygen affinity measurements of blood and hemolysate do not indicate biphasic oxygen saturation, showing that the functional properties of the variant are very similar to those of Hb A. This implies that the introduction of proline into the H-helix at position 138 does not disrupt the critical inter- and intrasubunit hydrogen bonds and salt bridges at the {beta} carboxyl-terminal dipeptide, since these polar interactions are essential for the normal oxygen-binding properties of hemoglobin. X-ray crystallographic data are consistent with these findings and show that the consequences of the {beta}138 Ala {yields} Pro substitution are almost entirely confined to the immediate vicinity of the mutation site. Instability probably results from the inability of a buried hydrogen bond to form between Pro 138{beta} and Val 134{beta}.

• OSTI ID: 6941073
• Journal Information: Biochemistry; (USA), Journal Name: Biochemistry; (USA) Vol. 27:20; ISSN 0006-2960; ISSN BICHA
• Country of Publication: United States
• Language: English

Related Subjects

550201* -- Biochemistry-- Tracer Techniques
59 BASIC BIOLOGICAL SCIENCES
ALANINES
AMINES
AMINO ACIDS
ANIMALS
AZOLES
CARBOXYLIC ACIDS
CHEMICAL BONDS
COHERENT SCATTERING
CRYSTALLOGRAPHY
DIFFRACTION
ELECTROPHORESIS
ELEMENTS
GLOBINS
HEMOGLOBIN
HETEROCYCLIC ACIDS
HETEROCYCLIC COMPOUNDS
MAMMALS
MAN
MUTATIONS
NONMETALS
ORGANIC ACIDS
ORGANIC COMPOUNDS
ORGANIC NITROGEN COMPOUNDS
OXYGEN
PIGMENTS
PORPHYRINS
PRIMATES
PROLINE
PROTEINS
PYRROLES
PYRROLIDINES
SCATTERING
VERTEBRATES
X-RAY DIFFRACTION