8-423-08AASynth - problem with the Phe hydroxylase HPA One...

Info icon This preview shows pages 1–7. Sign up to view the full content.

View Full Document Right Arrow Icon
1 Or, gluconeogenesis, FA synthesis AA breakdown AA synthesis
Image of page 1

Info iconThis preview has intentionally blurred sections. Sign up to view the full version.

View Full Document Right Arrow Icon
2 AA synthesis overview: -all are derived from intermediates of glycolysis, CAC, or PPP -Nitrogen enters by way of glutamate and glutamine -“families” of aas from six precursors -transamination reactions common -1-Carbon transfers common Serine, glycine synthesis -glycolytic precursor ( # ) -glutamate the N-donor, in a transamination reaction (<-----) -H 4 folate used to remove a 1-C group ( * ) (#from glycolysis) <----- *
Image of page 2
3 Transfers 1-C groups in intermediate oxidation states Transfers 1-C groups in most reduced state- methyl groups CH 4 Transfers 1-C groups in most oxidized state-CO 2 An essential aa Tyrosine synthesis -derived from the essential aa phenylalanine -Phe hydroxylase a mixed function oxidase
Image of page 3

Info iconThis preview has intentionally blurred sections. Sign up to view the full version.

View Full Document Right Arrow Icon
4 What if there is a
Image of page 4
Image of page 5

Info iconThis preview has intentionally blurred sections. Sign up to view the full version.

View Full Document Right Arrow Icon
Image of page 6
Image of page 7
This is the end of the preview. Sign up to access the rest of the document.

Unformatted text preview: problem with the Phe hydroxylase? Hyperphenylalaninemia HPA One severe type of HPA is Phenylketonuria PKU X Excessive levels of Phe build up Accumulation of aromatic metabolites of Phe in neural tissues 5 Excessive levels of Phe build up Accumulation of aromatic metabolites of Phe in neural tissues:-severe mental retardation In PKU: These compounds end up in urine, imparting a “mousy” odor 6 Glutamine made from glutamate and NH 4 + ; needs ATP Very highly regulated; it is the central entry point for nitrogen into metabolism Both allosteric modulation and reversible covalent modification used to regulate glutamine synthetase 7 Allosteric regulation Reversible covalent regulation...
View Full Document

{[ snackBarMessage ]}

What students are saying

  • Left Quote Icon

    As a current student on this bumpy collegiate pathway, I stumbled upon Course Hero, where I can find study resources for nearly all my courses, get online help from tutors 24/7, and even share my old projects, papers, and lecture notes with other students.

    Student Picture

    Kiran Temple University Fox School of Business ‘17, Course Hero Intern

  • Left Quote Icon

    I cannot even describe how much Course Hero helped me this summer. It’s truly become something I can always rely on and help me. In the end, I was not only able to survive summer classes, but I was able to thrive thanks to Course Hero.

    Student Picture

    Dana University of Pennsylvania ‘17, Course Hero Intern

  • Left Quote Icon

    The ability to access any university’s resources through Course Hero proved invaluable in my case. I was behind on Tulane coursework and actually used UCLA’s materials to help me move forward and get everything together on time.

    Student Picture

    Jill Tulane University ‘16, Course Hero Intern