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SRP5238

Sigma-Aldrich

PDHA1 (30-390), His tagged human

recombinant, expressed in E. coli, ≥70% (SDS-PAGE), buffered aqueous glycerol solution

Synonym(s):

PDHA, PDHCE1A, PHE1A

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About This Item

CAS Number:
1773497-23-6
UNSPSC Code:
12352200
NACRES:
NA.32

biological source

human

recombinant

expressed in E. coli

assay

≥70% (SDS-PAGE)

form

buffered aqueous glycerol solution

mol wt

~47 kDa

NCBI accession no.

NM_000284

application(s)

cell analysis

shipped in

dry ice

storage temp.

−70°C

Gene Information

human ... PDHA1(5160)

Biochem/physiol Actions

PDHA1 pyruvate dehydrogenase (lipoamide) alpha 1 is a member of the pyruvate dehydrogenase (PDH) complex. PDHA1 is a nuclear-encoded mitochondrial multienzyme complex which catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2), and provides the primary link between glycolysis and the tricarboxylic acid (TCA) cycle . PDHA1 plays a critical role in the brain that usually obtains all of its energy from the aerobic oxidation of glucose. Mutations in the PDHA1 are associated with pyruvate dehydrogenase E1-alpha deficiency and X-linked Leigh syndrome.

Physical form

Supplied in 50mM sodium phosphate, pH 7.0, 300mM NaCl, 150mM imidazole, 0.1mM PMSF, 0.25mM DTT, 25% glycerol.

Preparation Note

after opening, aliquot into smaller quantities and store at -70 °C. Avoid repeating handling and multiple freeze/thaw cycles

pictograms

Health hazardExclamation mark
GHS08,GHS07

signalword

Danger

Hazard Classifications

Eye Irrit. 2 - Repr. 1B - Skin Irrit. 2

Storage Class

6.1C - Combustible acute toxic Cat.3 / toxic compounds or compounds which causing chronic effects

wgk_germany

WGK 1

flash_point_f

Not applicable

flash_point_c

Not applicable

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Certificates of Analysis (COA)

Lot/Batch Number
I171-3

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K Chun et al.
Human molecular genetics, 2(4), 449-454 (1993-04-01)
Human PDH complex deficiency is an extremely heterogeneous disease in its presentation and clinical course. In an investigation at the level of the gene into ten cases of PDH complex (E1) deficiency, we found that all had mutations in the
Pyruvate dehydrogenase deficiency.
G K Brown et al.
Journal of medical genetics, 31(11), 875-879 (1994-11-01)

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