D-Bifunctional protein deficiency is an autosomal recessive peroxisomal fatty acid oxidation disorder. Peroxisomal disorders are usually caused by a combination...

D-bifunctional protein (DBP), also known as peroxisomal multifunctional enzyme type 2 (MFP-2), as well as 17β-hydroxysteroid dehydrogenase type IV (17β-HSD...

defects in single enzymes important for peroxisome function or in peroxins, proteins encoded by PEX genes that are critical for normal peroxisome assembly and...

hamster cells by separate transferase and decarboxylase proteins or by linker-deleted bifunctional protein". Somatic Cell and Molecular Genetics. 21 (3): 161–75...

This autosomal recessive disorder is caused by a deficiency in the enzyme UMPS, a bifunctional protein that includes the enzyme activities of OPRT and...

gangliosides. The two subunits of hexosaminidase A are shown below: The bifunctional protein NCOAT (nuclear cytoplasmic O-GlcNAcase and acetyltransferase) that...

"antibodies", were called by Ehrlich "amboceptors" to emphasise their bifunctional binding capacity: They recognise and bind to a specific antigen, but...

tissues, impaired fatty acid metabolism, anaemia, and systemic protein-energy deficiency. In contrast, an overabundance of lysine, caused by ineffective...

Perniola–Krajewska–Carnevale syndrome Perniosis Peroxisomal Bifunctional Enzyme Deficiency Peroxisomal defects Persistent Müllerian duct syndrome Persistent...

RNA (ncRNA) is a functional RNA molecule that is not translated into a protein. The DNA sequence from which a functional non-coding RNA is transcribed...

RE, Cygler M (February 1998). "The 3-D structure of a folate-dependent dehydrogenase/cyclohydrolase bifunctional enzyme at 1.5 A resolution". Structure...

a bifunctional enzyme that also exhibits phosphomannose isomerase (PMI) activity. Though not closely related to eukaryotic GPIs, the bifunctional enzyme...

for the protein, whereas in mammals and other multicellular organisms, the catalytic function is carried out by a domain of the bifunctional enzyme UMP...

Sourdive DJ, Transy C, Garbay S, Yaniv M (April 1997). "The bifunctional DCOH protein binds to HNF1 independently of its 4-alpha-carbinolamine dehydratase...

Sucrase-isomaltase is a bifunctional glucosidase (sugar-digesting enzyme) located on the brush border of the small intestine, encoded by the human gene...

In humans, deficiency of this enzyme results in a disease phenotype. MacKenzie RE, Aldridge M, Paquin J (10 October 1980). "The bifunctional enzyme...

genes in yeast, and related to, although not similar in structure, the bifunctional enzyme found in plants. In humans, mutations in the AASS gene, and the...

non-SMC element 4 homolog A OTUD1: encoding protein OTU deubiquitinase 1 PAPSS2: encoding enzyme bifunctional 3'-phosphoadenosine 5'-phosphosulfate synthase...

member 3 or SMAD3 is a protein that in humans is encoded by the SMAD3 gene. SMAD3 is a member of the SMAD family of proteins. It acts as a mediator of...

Darier disease; 124200; ATP2A2 Darsun syndrome; 612541; G6PC3 D-bifunctional protein deficiency; 261515; HSD17B4 De la Chapelle dysplasia; 256050; SLC26A2...

Bifunctional 3'-phosphoadenosine 5'-phosphosulfate synthetase 1 is an enzyme that in humans is encoded by the PAPSS1 gene. Three-prime-phosphoadenosine...

glycosylases: monofunctional and bifunctional. Monofunctional glycosylases have only glycosylase activity, whereas bifunctional glycosylases also possess AP...

and proteins necessary for transcription and subsequent translation. There are two main classes of glycosylases: monofunctional and bifunctional. Monofunctional...

concentration of Tailless and Huckebein proteins available to bind to the regulatory region. As a bifunctional transcription factor, Hunchback both activates...

(September 1981). "Purification of the putA gene product. A bifunctional membrane-bound protein from Salmonella typhimurium responsible for the two-step...

peptidoglycan. This is EC 2.7.7.23, also catalyzed by GlmU, which is a bifunctional enzyme. In step 5, some of the UDP-N-acetylglucosamine (UDP-GlcNAc) is...

is a bifunctional enzyme that cleaves the N-acetyl group from GlcNAc and subsequently sulfates the N-position. Next, C-5 uronyl epimerase coverts d-GlcA...

gene is a member of the aldehyde dehydrogenase family and encodes a bifunctional ATP- and NADPH-dependent mitochondrial enzyme with both gamma-glutamyl...

dehydrogenase deficiency Lowry-Wood syndrome Maturity-onset diabetes of the young type 6 Mitochondrial trifunctional protein deficiency Nonsyndromic deafness...

acutely leads to activation of protein phosphatase 2A (PP2A)[citation needed], which dephosphorylates the bifunctional enzyme fructose bisphosphatase-2...